Module 3

Hematology

Diagnostic Tests and Assessments

A. Red Blood cell count

Module 3

Hematology
Normal Laboratory Values for Red Blood Cells and Platelets

Laboratory Test

Normal Value

Red blood cell count

Men

4.2 to 5.4 million/mm3

Women

3.6 to 5.0 million/mm3

Reticulocytes

1.0% to 1.5% of total RBC

Hemoglobin
Men

14 to 16.5 g/dL

Women

12 to 15 g/dL

Module 3

Hematology
Normal Laboratory Values for Red Blood Cells and Platelets
Laboratory Test

Normal Value

Hematocrit
Men

40% to 50%

Women

37% to 47%

Mean Corpuscular
Volume (MCV)

85 to 100 fL/cell

Mean corpuscular
Hemoglobin concentration
(MCHC)

31 to 35 g/dL

Mean corpuscular
Hemoglobin (MCH)
Platelet count

27 to 34 pg/cell
250,000 to 400,000/mm3

Module 3

Hematology
Diagnostic Tests and Assessments
B. Hemoglobin and hematocrit
1. Hemoglobin - gas carrying
capacity of an erythrocyte
2. Hematocrit -ratio of the RBC
volume to the volume of whole
blood

Module 3

Hematology
Diagnostic Tests and Assessments
C. RBC Indexes
1. MCV (Mean corpuscular volume)estimate size of the RBC.
2. MCH (Mean corpuscular hemoglobin)measures the content of Hgb in RBCs
from a single cell
3. MCHC (Mean corpuscular hemoglobin
concentration)- a more accurate
measurement of the Hgb content of RBC
as it measures the entire volume of RBCs.

Module 3

Hematology
Diagnostic Tests and Assessments
D. Serum ferritin, transferrin, and
total iron-binding capacity (TIBC)Used to evaluate iron levels.
- Ferritin measures the iron in plasma,
which is also a direct reflection of total iron
stores.
- Transferrin is the major iron-transport
protein

Module 3

Hematology
Diagnostic Tests and Assessments
E. White Blood Cell Count
- *Abnormal elevation- leukocytosis
- Leukopenia is a decrease in the number of
white blood cells.

Module 3

Hematology
Table 14-2. Normal Laboratory Values: White Blood Cells

Laboratory Test

Value

WBC count

5000 to 10,000/mm3

Differential
Neutrophils

60 to 70% or 3000 to 7000/mm3

Eosinophils

1-3% or 50 to 400/mm3

Basophils

0.3 to 0.5% or 25 to 200/mm3

Lymphocytes

20 to 30% or 1000 to 4000/mm3

Monocytes

3 to 8% or 100 to 600/mm3

Module 3

Hematology
Diagnostic Tests and Assessments
F. Coagulation studies
Bleeding time normal range is 1- 4 minutes
- evaluation of platelet function
- Extended bleeding times thrombocytopenia and aspirin therapy

Module 3

Hematology
Diagnostic Tests and Assessments
F. Coagulation studies
Prothrombin Time (PT) is the rapidity of
blood clotting. Normal range is 11-16
seconds.
*PT evaluates extrinsic coagulation
pathway which include
- Factor I,

- Factor VII,

- Factor II,

- Factor X.

- Factor V,

Module 3

Hematology
Diagnostic Tests and Assessments
F. Coagulation studies
Partial thromboplastin time (PTT) normal range is 6070 seconds, which *evaluates the intrinsic coagulation
pathway or fibrin clot formation.
Activated partial thromboplastin time (APTT) normal
range is 30-45 seconds.
- Used in heparin therapy and in the evaluation of hemophilia.
- APTT is increased in
anticoagulation therapy,
liver disease,
vitamin K deficiency,
disseminated intravascular coagulation. (DIC).

Module 3

Hematology
Diagnostic Tests and Assessments
F. Coagulation studies
Fibrinogen normal range is 150-400 mg/dl . It is a
soluble plasma protein that is decreased in DIC
Fibrin degradation products (FDP): normal value is
< 10 micrograms/ml. FDP is increased in fibrinolysis,
thrombolytic therapy, and DIC.
Fibrin D-dimer - normal is 0 - 0.5 micrograms/ml. Ddimer is the most sensitive indicator to differentiate DIC
from primary fibrinolysis. It is.elevated in DIC.

Module 3

Hematology
Diagnostic Tests and Assessments
G. Bone Marrow Examination
Sites for bone marrow aspiration may include:
- sternum,
- iliac crest (most common),
- Tibia.
- The most common site posterosuperior iliac spine.

Module 3

Hematology
Diagnostic Tests and Assessments
H. Lymphangiography is visualization
of the lymph system radiographically
after injection of a dye. (staging of
Hodgkins and non-Hodgkins
lymphoma).

Module 3

Hematology
Diagnostic Tests and Assessments
H. Lymph Node Biopsy performed in
the operating room to obtain lymph
tissue for histologic analysis.

Module 3

Hematology

Common nursing
procedures of the
hematologic system

Module 3

Hematology
Common nursing procedures of the hematologic system
A. Protocol for the administration of blood and blood products

1. Check the agencys policy and procedure.

2. Verify the physicians order

Module 3

Hematology
Common nursing procedures of the hematologic system
A. Protocol for the administration of blood and blood products
3. Consent
4. Typing and crossmatch .
5. Obtain blood intravenous access line is available.
6. IV with normal saline
7.*Blood may not be returned to the blood bank after 20 minutes.
8. Do not keep blood or blood products in the nursing unit
refrigerator.

Module 3

Hematology
Common nursing procedures of the hematologic system
A. Protocol for the administration of blood and blood products
9. Validate data of blood product with another nurse.
10. Validate with another nurse that the clients name, ID number,
blood type and Rh matches the unit of blood to be transfused.
11. Note the expiration date indicated on the blood product
12. Observe the unit of blood for bubbles or discoloration.

Module 3

Hematology
Common nursing procedures of the hematologic system
A. Protocol for the administration of blood and blood products
13. Pre-transfusion vital signs, 15 minutes after the
transfusion is initiated and immediately after the
completion of the transfusion.
14. *A pre-transfusion temperature of 100 F should be
reported before initiating the transfusion. Any increase in
2 degrees F in the clients temperature may be an
indication of a transfusion reaction
15. *Start blood transfusion slowly (25-50 mL during the
first 15 minutes). Stay with the client during first 15 min.

Module 3

Hematology
Common nursing procedures of the hematologic system
A. Protocol for the administration of blood and blood products
16. Do not administer any medications through the blood
transfusion tubing.
17. Use only agency- approved blood- warming devices.
18. Blood products should not be infused longer than 4 hours.
19. Discard tubings and bags used in the transfusion in a
biohazard receptacle.
20. Follow protocol for transfusion reactions.

Module 3

Hematology
Common nursing procedures of the hematologic system
B. Protocol for suspected blood transfusion reaction

1. Check agency protocol.

2. Stop the infusion immediately. Change IV
tubing and keep vein open with normal saline.
3. Assess the client for other signs and symptoms
of transfusion reaction.

Module 3

Hematology
Common nursing procedures of the hematologic system
Signs and Symptoms of Blood Transfusion Reaction
Hemolytic
- Chills,
- fever,
- urticaria,
- tachycardia,
- chest pain or
complaints of chest
tightness,
- shortness of breath,

- dyspnea,
- lumbar pain,
- nausea,
- vomiting,
- rales,
- hematuria,
- hypotension,
- wheezing.

Module 3

Hematology
Common nursing procedures of the hematologic system
Signs and Symptoms of Blood Transfusion Reaction
Bacterial (Pyrogenic)
- Hypotension,
- fever,
- chills,
- flushed skin,
- abdominal pain,
- pain in extremities,
- vomiting,
- diarrhea.

Module 3

Hematology
Common nursing procedures of the hematologic system
Signs and Symptoms of Blood Transfusion Reaction
Allergic reaction
- Urticaria,
- pruritus,
- swelling of the tongue,
- swelling of the face,
- difficulty of breathing,
- pulmonary edema,
- shock.

Module 3

Hematology
Common nursing procedures of the hematologic system
Signs and Symptoms of Blood Transfusion Reaction
Circulatory Overload
- Chest pain,
- tightness of the chest,
- cough,
- rales,
- pulmonary edema,
- tachycardia,
- elevated blood pressure.

Module 3

Hematology
Common nursing procedures of the hematologic system
B. Protocol for suspected blood transfusion reaction
4. Notify the physician and the blood bank.
5. Send the unit of blood and tubing used to the
blood bank.
6. Urine and blood samples will be required.
7. Administer prescribed drugs
8. Document the reaction and interventions.

Module 3

Hematology
Common Disorders of the hematologic system

A. Iron deficiency Anemia (IDA)

1. Description:
Supply of iron is inadequate for optimal
formation of RBCs related to
excessive iron loss due to bleeding,
decreased dietary intake,
malabsorption.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

2. Etiology and pathophysiology:

- Accounts for 60% of anemias in clients over age 65.
-Most common cause -blood loss from
gastrointestinal or genitourinary system.
- Normal iron excretion is less than 1 mg/day through
the urine, sweat, bile, feces, and from desquamated
cells of the skin;
- 0.5 mg of iron daily or 15 mg monthly during
menstruation; .

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

2. Etiology and pathophysiology:

- Reduced oxygen carrying capacity of the blood,
producing tissue hypoxia.
-Iron is stored in the body as ferritin,
-it is formed in the intestinal mucosa, when ferritin
iron joins with the protein apoferritin;
-*ferritin is stored in the tissues, primarily in the
reticuloendothelial cells of the liver, spleen, and
bone marrow.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

2. Etiology and pathophysiology:

-Develops slowly through three phases:
-bodys stores of iron depleted, insufficient iron is
transported to the bone marrow and iron deficient
erythopoiesis begins, small hemoglobin deficient cells
enter the peripheral circulation in large numbers; iron is
needed on the hemoglobin so the oxygen molecule will
attach.
- Adequate iron in the RBC is essential since the
oxygen molecule attaches to it.
- An average diet supplies the body with 12 to 15
mg/day of iron, of which only 5-10% is absorbed.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

3. Assessment
a. Clinical manifestations (usually develop gradually with
the client not seeking attention until the hemoglobin
drops to 7-8 g/dL).
- Fatigue
- Cheilosis (cracks in the
corners of the mouth)
- Weakness
- Smooth sore tongue
- Shortness of breath
- Dizziness
- Pallor (ear lobes, palms
- Pica (craving to eat
and conjunctiva)
unusual substances such
- Brittle spoon like nails
as clay or starch).

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

3. Assessment

b. Diagnostic and laboratory tests

- * Microcytic-hypochromic anemia (RBC diameter <6
with decreased pigmentation)
- Increase in the red cell size distribution width (RDW).
- Erythrocytes are small (microcytic) and pale
(hypochromic);
- Mean corpuscular volume (MCV; measures size) is
decreased

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

3. Assessment
b. Diagnostic and laboratory tests
- MCH or MCHC decreased;
- The MCV, MCH, and MCHC should be analyzed
only when the hemoglobin is low.
- Low serum iron level and elevated serum ironbinding capacity or low serum ferritin levels.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

4. Therapeutic management:
- The cause for the anemia is usually explored.
- Stools are examined for occult blood.
- Endoscopic examination
- Increase intake of iron-rich foods
- Vitamin supplementation. Administration of oral iron
preparation in the form of ferrous sulfate.
- Parenteral administration of iron.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

Sources of Dietary Iron

- Organ meat
- Meat
- Green leafy vegetables
- Beans
- Molasses
- Raisin

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

5. High priority nursing diagnosis:

- Activity intolerance;
- Risk for decreased cardiac output;

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

6. Planning and implementation:

- *Administer oral iron preparation with orange juice or
vitamin C to increase absorption. Antacids interfere
with the absorption of iron.
- *Administer parenteral iron deep intramuscularly
via the Z tract method.
- Identify/implement energy saving techniques,
e.g., shower chair, sitting to perform tasks.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

6. Planning and implementation:

- *Promote quiet environment to facilitate sleep/rest.
- Monitor for dizziness, suggest position changes
be made slowly.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

6. Planning and implementation:

- *Encourage/assist with good oral hygiene before and
after meals, using soft bristled toothbrush for gentle
brushing of fragile gums.
- Determine stool color, consistency, frequency,
and amount.
- Encourage fluid intake of 2500-3000 ml day.
- Discuss use of stool softeners, bulk forming
laxatives, mild stimulants or enemas if indicated.
Monitor effectiveness.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

6. Planning and implementation:

- *Oral liquid form of iron can stain the teeth. Use a
straw or place spoon at the back of the mouth to take
the supplement and rinse mouth thoroughly afterward.
- Caution that bowel movement may appear
greenish black/tarry.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

6. Planning and implementation:

- *Deep IM, Z-track administration of medication. Use
separate needles for withdrawing and injecting the
medication.
- Caution -systemic (allergic) reactions (flushing,
nausea/vomiting, myalgias) and the importance of
reporting symptoms.
- Refer to social services for food stamps, meals
on wheels.

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

7. Medication therapy
- Oral ferrous sulfate (FeSO4) 300-325 mg tid, given 1
hr before meals for 6 months.
- ferrous gluconate (Fergon) and ferrous fumarate
(Ircon, Femiron).
- Iron dextran (INFeD) deep IM (Z-track) or IV
therapy may be given. A small test dose is
administered.
- Transfusion of packed RBCs

Module 3

Hematology
Common Disorders of the hematologic system
A. Iron deficiency Anemia (IDA)

8. Client education
- maintain good nutrition
- *take iron on an empty stomach. Absorption of
iron is decreased with food.
- *stools will appear black with the oral intake of
iron.
- *prevent constipation.

Module 3

Hematology
Common Disorders of the hematologic system

B . Megaloblastic anemia
1. Vitamin B12 deficiency anemia
- Description: A type of anemia
characterized by macrocytic red blood
cells.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- Etiology and pathophysiology:
- *Inevitably develops after total gastrectomy or
gastrojejunostomy.
- *Lack of vitamin B12 alters the structure and disrupts the
function of the peripheral nerves, spinal cord and brain.
- *Lack of Vitamin B12 impairs cellular division and
maturation especially in rapidly proliferating RBC's.
- *Pernicious anemia is the bodys inability to absorb
Vitamin B12 due to a lack of intrinsic factor, a substance
secreted by the parietal cells of the gastric mucosa.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- Assessment
Clinical manifestations: Signs and symptoms include

- pallor or slight jaundice

- complaint of weakness,
- * smooth sore beefy red tongue (glossitis),
- diarrhea,
- paresthesias
- Difficult prioception
- fair haired or prematurely gray.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- Diagnostic and laboratory tests:
- increase in the MCV and MCHC
- Gastric secretion analysis reveals
achlorhydria: the absence of free
hydrochloric acid in a pH maintained at 3.5.
- *24 hour urine for Schilling test confirms
pernicious anemia

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- Therapeutic management:
-Dietary counseling
- If deficiency caused by a vegetarian diet,
fortified soy milk may be added to the diet or oral
supplements of B12 may be added.
- If to gastric malabsorption, life long replacement
therapy is required. Intramuscular injection of B12
is required and sometimes megadoses of oral
vitamins may be given.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- High priority nursing diagnosis:
- High risk for injury;
- Activity intolerance;
- Altered oral mucous membrane.

Module 3

Hematology
Common Disorders of the hmatologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- Planning and implementation:
- assess for neurologic deficits
- interventions relating to prevention
of injury.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- Medication therapy
- Parenteral vitamin B12 , 100-1000
micrograms subcutaneously daily for 7
days, then once a week for 1 month,
then monthly for the remainder of life

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

1. Vitamin B12 deficiency anemia

- Client education
- Burning sensation felt after a parenteral dose
of Vitamin B12 is temporary.
- Dietary sources of Vitamin B12 like dairy
products, animal proteins, and eggs.
- For pernicious anemia, discuss regular
parenteral Vitamin B12 and importance of
continued treatment.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Etiology and pathophysiology
- poor nutrition,
- malabsorption syndrome,
- medications that impede the absorption (oral
contraceptives, anticonvulsants, methotrexate
[MTX]),
- alcohol abuse
- anorexia.
- *Alcoholics and those receiving total parenteral
nutrition are at risk.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Etiology and pathophysiology
- *Pregnant women, infants and teenagers are
also at risk
- *Clients on hemodialysis.
- Lack of folic acid causes the formation of
megaloblastic cells. These cells are fragile.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Assessment:
Clinical manifestations:

- pallor,
- progressive weakness,
- fatigue,
- shortness of breath,
- cardiac palpitations;
- GI symptoms are similar to B12 deficiency, but
usually more severe (glossitis, cheilosis, and
diarrhea);
- **neurological symptoms seen in B12
deficiency are not seen in folic acid deficiency
and therefore assist in the differentiation of
these two types of anemia.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Diagnostic and laboratory tests
- Macrocytic (megaloblastic) anemia
(RBC diameter > 8);
- MCV high with low hemoglobin.
- Low serum folate level

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Therapeutic management
- dietary counseling
- administration of folic acid.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

High priority nursing diagnosis:
- Activity intolerance;
- Constipation;
- Diarrhea;

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Planning and implementation
- energy saving techniques, e.g., shower chair,
sitting to perform tasks
- Monitor for dizziness, suggest position changes be
made slowly.
- Encourage/assist with good oral hygiene before and
after meals, using soft bristled toothbrush for gentle
brushing of fragile gums.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Medication therapy
- Oral folate, 1-5 mg/day for 3-4
months.
- Folate should be given along with
vitamin B12 when both are deficient.

Module 3

Hematology
Common Disorders of the hematologic system
B . Megaloblastic anemia

2. Folic acid deficiency anemia

Client education
- *Dietary sources of folic acid - green leafy
vegetables, fish, citrus fruits, yeast, dried
beans, grains, nuts, and liver.
- Teach clients at risk (alcoholics, clients on
hemodialysis and certain drugs) to increase
their dietary intake through diet selection and
supplementation.
- strategies to decrease pain associated with
glossitis such as eating bland and soft foods.

Module 3

Hematology
Common Disorders of the hematologic system

C . Aplastic anemia
1. Description
Aplastic anemia is a form of anemia
resulting in decreased production of
bone marrow elements namely
erythrocytes, leukocytes, and platelets.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia
2. Etiology and pathophysiology
a. Affects all age groups and gender.
b. Two classifications
- Congenital aplastic anemia is caused by a
chromosomal alteration.
-Acquired form may be caused by radiation,
chemical agents and toxins, drugs, viral and
bacterial infections, pregnancy, and idiopathic.
-In about 50% of cases, the cause is unknown.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia
2. Etiology and pathophysiology
c. There is a decrease or cessation of
production of RBCs (anemia), WBCs
(leukopenia), and platelets
(thrombocytopenia). Decrease may result
from damage to bone marrow stem cells, the
bone marrow itself, and the replacement of
bone marrow with fat. Condition may be
acute or chronic.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia
3. Assessment
- Clinical manifestations
- Pallor
- Fatigue
- Palpitations
- Exertional dyspnea
- Infections of the skin and mucous
membranes
- Bleeding from gums, nose,
vagina, or rectum.
- Purpura (bruising)
- Retinal hemorrhage

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia

3. Assessment
- Diagnostic and laboratory tests
- *Blood counts reveal pancytopenia
(decreased RBC, WBC, and platelets).
- Decreased reticulocyte count.
- Bone marrow examination reveals
decrease in activity of the bone
marrow or no cell activity.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia

4. Therapeutic management
- Identification of the cause of bone marrow
suppression
- Bone marrow transplantation.
- Immunosuppression
- Transfusion of leukocyte-poor RBCs.
- Spleenectomy.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia

5. High priority nursing diagnosis

- High risk for infection;
- High risk for bleeding;
- Activity intolerance.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia

6. Planning and implementation

- Institute reverse isolation.
- Limit visitors and potential sources of infection
- Monitor for evidence of bleeding.
- Avoid invasive procedures including rectal
temperatures
- Provide frequent rest periods
- Monitor tolerance to activities.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia

7. Medication therapy
- Agents that suppres lymphocyte activity
such as antilymphocyte globulin (ALG),
antithymocyte globulin (ATG) and
cyclosporine (Sandimmune).
- Immunosuppresive agents such as
prednisone and cyclophosphamide (Cytoxan).

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia

8. Client education
- preventing infection
- prevent hemorrhage
- avoidance of drugs that increase bleeding
tendency such as aspirin.
- balance activity with adequate rest periods to
avoid fatigue.
- report signs of infection, bleeding, increasing
intolerance to activity.

Module 3

Hematology
Common Disorders of the hematologic system
C . Aplastic anemia

9. Evaluation
- Client verbalizes ways to prevent infection,
bleeding, and fatigue.
- Does not develop infection.
- Does not develop hemorrhage.

Module 3

Hematology
Common Disorders of the hematologic system

D . Sickle cell disease

1. Description:
- a hereditary, chronic
form of hemolytic anemia.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease

2. Etiology and pathophysiology

- Eight percent of African-Americans are
heterozygous (carriers) for sickle cell anemia
thereby inheriting one affected gene or the
sickle cell trait.
- One percent of African-Americans are
homozygous (identical genes) for the
disorder, thereby inheriting a defective gene
from both parents or sickle cell anemia and
are likely to experience sickle cell crisis.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease
2. Etiology and pathophysiology
- *Sickle cell trait (heterozygous state) is a
generally mild condition that produces few, if any
manifestations.

- Sickle cell anemia is caused by an autosomal

genetic defect (one gene affected) that results in
the synthesis of hemoglobin S.
- Produced by a mutation in the beta chain of the
hemoglobin molecule though a substitution of the
amino acid valine for glutamine in both beta
chains.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease
2. Etiology and pathophysiology
- During decreased oxygen tension in the plasma,
the hemoglobin S causes the RBCs to elongate,
become rigid, and assume a crescent sickled
shape. Cells clump, obstruct capillary blood flow
causing ischemia and possible tissue infarction:

- *Conditions likely to trigger a sickle cell

crisis include: hypoxia, low environmental and/or
body temperature, excessive exercise, high
altitudes, or inadequate oxygen during
anesthesia.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease
2. Etiology and pathophysiology
- Other causes of sickle cell crisis include:
- elevated blood viscosity/decreased plasma volume,
- infection,
- dehydration,
- and/or increased hydrogen ion concentration (acidosis).

- With normal oxygenation, the sickled RBCs

resume their normal shape. Repeated episodes of
sickling and unsickling weaken the cell membrane,
causing them to hemolyze and be removed.

- Crisis is extremely painful and can last from 4-6 days.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease

3. Assessment
Clinical manifestations
- Pallor
- Jaundice
- Fatigue
- Irritability
- Large joints and surrounding tissue
may become swollen during crisis
- Priapism

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease

3. Assessment
Diagnostic and laboratory tests
- Anemia with sickled cells noted on a
peripheral smear.
- Hemoglobin electrophoresis to determine the
presence and percentage of hemoglobin S is
used for a definitive diagnosis.
- Elevated serum bilrubin levels.
- Elevated reticulocyte count.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease

4. Therapeutic management
- Bone marrow transplantation
- Blood transfusions
- Management of pain
- Use of chemotherapy drug
hydroxyurea (Droxia) to increase
hemoglobin F and decrease sickling

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease

5. High priority nursing diagnosis:

- Pain;
- Tissue perfusion, altered;
- Gas exchange, impaired;

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease

6. Planning and implementation

- Teach prevention of sickle cell crisis.
- Referral to appropriate agency for
genetic counseling and family planning.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease
Clients who are in crisis should have the following included

- Management of pain.
- Administration of oxygen.
- *Promoting hydration to decrease blood
viscocity. The client in crisis should have
an oral intake of at least 6 to 8 quarts per
day or IV fluids of 3 liters per day.

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease
Monitor for complications such as
- vaso-occlusive disease (thrombosis),
- hypoxia,
- CVA,
- renal dysfunction,
- priapism leading to impotence,
- acute chest syndrome (fever, chest pain, cough,
pulmonary infiltrates, and dyspnea),
- substance abuse.
Management of infection

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease

7. Medication therapy:
- * Nifedipine (Procardia) may be used
for priapism.
- Hydroxyurea (Droxia) to increase
hemoglobin F and decrease sickling
- Narcotic analgesics during the acute
phase of sickle cell crisis.
- Broad spectrum antibiotics to manage
acute chest syndrome.
- Folic acid supplements

Module 3

Hematology
Common Disorders of the hematologic system
D . Sickle cell disease
Teach client ways to prevent sickle cell crisis
- *Maintaining adequate fluid intake. Clients with sickle cell
disease should maintain an oral intake of at least 4 to 6
quarts per day. Avoid conditions that might predispose them
to dehydration.
- Avoiding high altitudes
- Prevention of and prompt treatment of infections
- Stress reduction strategies
- Avoid exposure to cold.
- Avoid overexertion.
- Adhere to vaccination schedules for pneumococcal
pneumonia, haemophilus influenza type B, and hepatitis B.
- Importance of regular medical follow-up.

Module 3

Hematology
Common Disorders of the hematologic system

E . Polycythemia
- an increase in the number of circulating
erythrocytes and the concentration of
hemoglobin in the blood; also known as
polycemia vera, PV, or Myeloproliferative
red cell disorder.
- Polycythemia can be primary or secondary

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Etiology and pathophysiology
a. Primary
- Common in men of European Jewish descent.
- Neoplastic stem cell disorder characterized by
increased production of RBCs, granulocytes, and
platelets.
- With the over production of erythrocytes, there is
increased blood viscocity resulting in congestion of
blood in tissues, the liver, and spleen.
- Thrombi form, acidosis develops, and tissue infraction
occurs as a result of the diminished circulatory flow of
blood due to the increased viscosity.

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Etiology and pathophysiology
b. Secondary
- Most common form of polycythemia vera.
- The disturbance is not in the development of red blood cells
but in the abnormal increase of ertythropoietin, causing
excessive erythropoiesis.
- The increase in red blood cell production due to increased
erythropoietin release is a physiologic response to hypoxia.
Hypoxia stimulates the release of erythropoietin in the kidney.
- Chronic hypoxic states may be produced by prolonged
exposure to high altitudes, pulmonary diseases,
hypoventilation, and smoking.
- The results of an increased RBC production include the
increased viscosity of blood, which alters circulatory flow.

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Assessment
Clinical Manifestations
- Plethora- a ruddy (dark, flushed) color of the face,
hands, feet, ears, and mucous membranes resulting
from the engorgement or distention of blood vessels.
- Symptoms associated with increased blood volume including
headaches, vertigo, blurred vision, and tinnitus.
- Distended superficial veins.
- Itching unrelieved by antihistamines.
- Symptoms associated with impaired tissue oxygenation
including angina, claudication, or dyspnea.
- Erythromyalgia, or burning sensation of the fingers and toes.
- Splenomegaly in majority of those with primary polycythemia vera.
- Epistaxis, GI bleeding

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Diagnostic and Laboratory tests:
- Elevated hemoglobin and erythrocyte count
- Decreased MCHC
- Increased WBC and basophilia
- Increased platelets
- Elevated leukocyte alkaline phosphatase
- Elevated uric acid
- Elevated cobalamin levels
- Increased histamine levels
- Bone marrow examination shows hypercellularity.

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Therapeutic management
- Management of the underlying condition
(such as COPD) causing the chronic hypoxia.
- *Repeated phlebotomy to decrease blood
volume. The goal is to keep the hematocrit
less than 45 to 48%.
- Hydration to decrease blood viscosity.

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia

High priority nursing diagnosis

- Impaired gas exchange;
- Pain;
- Risk for infection

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Planning and implementation
- Assist in phlebotomy
- Measures to relieve pruritus including cool
and tepid baths
- Accurate monitoring of fluid intake and output
- Nursing measures to prevent thrombotic
events including early ambulation, passive leg
exercises when on bed rest, avoid crossing
legs, and maintaining adequate hydration
- Administration of medications for the prevention of
complications including anticoagulants.

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Medication therapy
- Myelosuppressive agents to inhibit bone
marrow activity including hydroxyurea
(Hydrea), melphalan (Alkeran) and radioactive
phosphorous
- Allopurinol to manage gout.
- Antiplatelet agents to prevent thrombotic
complications

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia
Client education
- *importance of maintaining good hydration. The
client should drink at least 3 liters of fluid per day.
(Hydrea), melphalan (Alkeran) and radioactive
phosphorous
- about the disease and ways in which it can be
controlled, such as smoking cessation.
- Discuss signs and symptoms of complications
associated with the disorder
- prevent bleeding states
- importance of a regular medical check up.
- avoid products that contain iron.
- ways of preventing thrombosis.

Module 3

Hematology
Common Disorders of the hematologic system
E . Polycythemia

Evaluation
- The hematocrit is within normal range.
- The client does not develop complications
associated with thrombus formation.
- The client maintains adequate hydration.

Module 3

Hematology
Common Disorders of the hematologic system

F . Thrombocytopenia
Definition:
A decrease in the number of circulating
platelets or a platelet count of less than
100,000 platelets per milliliter of blood
resulting in problems of hemostasis.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Etiology and pathophysiology

- The decrease in the number of
circulating platelets may be a result of
three mechanisms: decreased
production, increased destruction, or
increased consumption.
- The cause of decreased production of
platelets may be inherited or acquired.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia
Etiology and pathophysiology
- Increased destruction of platelets may be
caused by an immune system defect. The
platelets become coated with an antibody.
- When these antibody coated platelets reach the
spleen, they are recognized as foreign and are
destroyed.
- Platelets normally have a circulating life of 8 to 10
days but because of this immune response their life
cycle is shortened. This condition is referred to as
immune thrombocytopenic purpura (ITP)

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia
Etiology and pathophysiology
- The acute form of ITP is more common in children
whereas the chronic form is more common in
women between the ages of 20 to 50.
- Other causes of increased destruction of platelets
include non -immune related factors such as
infection or drug induced effects.
- A decrease in the number of functional platelets
leads to bleeding disorders. Cerebral and
pulmonary hemorrhage can occur when platelet
counts drop below 10,000/mm3.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Assessment
Clinical manifestations
- Petechiae and purpura (anterior thorax,
arms, and neck).
- Epistaxis, gingival bleeding,
menorrhagia, hematuria, and
gastrointestinal bleeding.
- Signs of internal hemorrhage

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Diagnostic and laboratory tests

- Decreased hemoglobin and hematocrit if
bleeding is present.
- Decreased platelet count.
- Prolonged bleeding time.
- Bone marrow examination to determine the
etiology. May reveal decreased platelet
activity or increased megakaryocytes.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Therapeutic management
- Treatment of the underlying cause or removal
of the causative agent.
- Use of immunosuppressive and
chemotherapeutic agents in cases of ITP
- Platelet transfusions if there is active
bleeding.; little benefit in ITP.
- Splenectomy in ITP

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

High priority nursing diagnosis

- Injury, risk for bleeding;
- Fatigue;
- Altered oral mucous membrane

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Planning and implementation

- *Institute bleeding (thrombocytopenic) precautions:
- Avoid intramuscular or subcutaneous injections
- Avoid indwelling catheters
- If absolutely necessary use smallest gauge
needles for injections or venipunctures.
- Apply pressure on injection sites for 5 minutes or
until bleeding stops.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Planning and implementation

- *Institute bleeding (thrombocytopenic) precautions:
- Discourage straining at stool, vigorous coughing and
nose blowing.
- Avoid rectal manipulation such as rectal temperatures,
suppositories, or enemas.
- Discourage the use of razors. Use only electric
- Use soft-bristled toothbrush or toothettes and avoid
shavers.
flossing.
- Pad side rails if necessary and avoid tissue trauma
- Avoid the use of aspirin and drugs that interfere with
blood coagulation.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Planning and implementation

- Monitor for signs of bleeding. Test stools
for occult blood.
- Monitor CBC and platelet counts.
- Administer platelets as ordered.
- Monitor response to therapy.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Medication therapy
- Steroids and immunoglobulins may be used
to suppress the immune response in ITP
- Immunosuppressive agents may be used such
as vincristine (Oncovin) and cyclophosphamide
(Cytoxan).
- Platelet growth factor such as oprelvekin
(Neumega).

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Client education
- The client and family should be taught to
monitor for signs of bleeding and when to
contact the primary care provider.
- Instructions on bleeding precautions including
- use of soft-bristled toothbrush
- avoidance of flossing
- prevention of tissue trauma and injury including
vigorous sexual intercourse,
- using an electric razor for shaving.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Client education
- Teach client to avoid drugs that contain
aspirin and others that interfere with
coagulation.
- Discuss medication dosing, schedule,
and side effects.
- Teach the importance of regular medical
follow up and platelet monitoring.

Module 3

Hematology
Common Disorders of the hematologic system
F . Thrombocytopenia

Evaluation
- There is no evidence of bleeding.
- Increased platelet count.
- Client verbalizes knowledge of
medication actions and precautions.
- Client identifies methods to monitor for
signs of occult bleeding

Module 3

Hematology
Common Disorders of the hematologic system

G . Hemophilia
Description
- A group of hereditary clotting factor
disorders characterized by prolonged
coagulation time that results in prolonged
and sometimes excessive bleeding.

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Etiology and pathophysiology

- Hemophilia A and B are X-linked recessive disorders
transmitted by female carriers, displayed almost
exclusively in males.
- Hemophilia A (classic hemophilia) is a deficiency in
Factor VIII . Most common form of hemophilia.
- Hemophilia B (Christmas disease) is a deficiency in
Factor IX.
- Hemophilia A & B are clinically identical.
- In clients with hemophilia A &B, platelet plugs are
formed at the site of bleeding, but the clotting factor
impairs the coagulation response and the capacity to
form a stable clot.

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Etiology and pathophysiology

- Von Willebrands Disease is a related
disorder caused by a deficiency of the von
Willebrands factor (vWF), which is necessary
for factor VIII activity and platelet adhesion.
This disorder affects men and women equally

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Assessment

Clinical manifestations
- Persistent and prolonged bleeding from small cuts
and injuries.
- Delay of onset of bleeding after an injury.
- Subcutaneous ecchymosis and subcutaneous
hematomas.
- Gingival bleeding
- Gastrointestinal bleeding.
- Hematuria
- Pain, paresthesias, or paralysis resulting from nerve
compression of the hematomas.
- Hemarthrosis

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Diagnostic and laboratory tests

- Specific factor assays to determine the type
of hemophilia present
- APTT is increased in all types of hemophilia
- Bleeding time is prolonged in von
Willebrand disease
- Decreased factor VIII in hemophilia A, vWF
disease, and factor IX in hemophilia B.

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Therapeutic management
- Treatment is the replacement of the
deficient coagulation factor(s).
- Hemophilia A: cryoprecipitate containing 8100 units of factor VIII per bag at 12- hour
intervals until bleeding ceases. Freeze-dried
concentrate of Factor VIII may also be given.
- Hemophilia B: plasma or factor IX
concentrate given Q 24 hours or until
bleeding ceases.

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia
Therapeutic management
- Von Willebrands disease: cryoprecipitate
containing 8-100 units of factor VIII per bag at 12hour intervals until bleeding ceases.
Desmopressin (DDAVP) given intravenously may
also be used.
- Supportive treatment for hemarthrosis including
arthrocentesis and physiotherapy.
- Control of topical bleeding with hemostatic
agents, pressure, and application of ice.
- Management of complications associated with
hemorrhage.

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

High priority nursing diagnosis:

- Risk for injury, bleeding;
- Decreased cardiac output;
- Fluid volume deficit;
- Risk for ineffective management of
therapeutic regimen.

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Planning and implementation

- Teach the client and family about the disease and

therapeutic regimen
- Refer for genetic counseling and family planning.
- Refer to the National Hemophilia Foundation for
support and counseling
- *Monitor for signs of complications including
hemarthrosis and intracranial bleeding
- Assist in the management of pain associated with
hemarthrosis
- Control bleeding and maintain hemostasis
- Administer medications as prescribed.

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Client education

- Signs and symptoms requiring immediate

medical attention
- *Precautions to prevent bleeding including.
- Medic Alert bracelet indicating about the
hemophilia.
- *Maintain good dental hygiene to decrease
the necessity of invasive dental procedures.
- Adhering to scheduled visits and follow up
care

Module 3

Hematology
Common Disorders of the hematologic system
G . Hemophilia

Evaluation
- The client identifies strategies to prevent
injury and bleeding precautions.
- There is no evidence of internal bleeding
including hemarthrosis.
- *The client and family seek support from the
local chapter of the National Hemophilia
Society.

Module 3

Hematology
Common Disorders of the hematologic system

H . Disseminated intravascular
coagulation (consumption coagulopathy)
Description
is a syndrome characterized by abnormal
initiation and acceleration of clotting and
simultaneous hemorrhage. The paradoxical
bleeding that occurs is a result of the
consumption of clotting factors and platelets.
The syndrome is usually precipitated by an
underlying pathologic condition.

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Etiology and pathophysiology

- Mortality rate associated with DIC is as high
as 80% with the most frequent sequela being
hemorrhage.
- The syndrome is precipitated by conditions
such as widespread tissue damage,
hemolysis, hypotension, hypoxia, and
metabolic acidosis.

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Risk Factors for DIC

- Venomous snakebite

- Tissue necrosis

- Sepsis
- Trauma

- Drug reactions

- Obstetric complications

- Liver disease

- Neoplasms

- Acute hemolysis

- Vascular disorders

- Extensive burns

- Hypoxia

- Prosthetic devices

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Etiology and pathophysiology

- *The underlying condition causes initiation and

widespread formation of clots in the vascular system
either through the activation of factor XII, factors II and
X, or the release of tissue thromboplastin. Substances
necessary for clotting are used at a more rapid rate than
it can be replaced.
- As the clotting continues, the fibrinolytic pathway is
activated to dissolve the clots formed. Clotting factors
become depleted while fibrinolysis continues. Platelets
decrease, clotting factors II, V, VIII, and fibrinogen are
depleted

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Etiology and pathophysiology

- Fibrin degradation products (FDP) are released as a
result of fibrinolysis. Fibrin degradation products (FDP),
which are potent anticoagulants used to lyse the clots
further increases the bleeding state.
- With the depletion of clotting factors and the increase
in fibrin degradation products stable blood clots no
longer form and hemorrhage occurs.

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

Assessment

Clinical Manifestations of DIC

Integumentary
- Decreased skin temperature
- Pallor
- Purpura
- Ecchymoses
- Hematomas
- Acral cyanosis
- Superficial gangrene
- Altered sensation
- Gingival bleeding
- Bleeding from puncture sites

(consumption coagulopathy)

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Assessment
Clinical Manifestations of DIC

Gastrointestinal
- Hemoptysis
- Melena
- Occult blood in stool or vomitus
- Abdominal distention
- Abdominal pain

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

Assessment
Clinical Manifestations of DIC

Respiratory
- Dyspnea
- Tachypnea
- Orthopnea
- Decreased breath sounds
- Chest pain

(consumption coagulopathy)

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Assessment
Clinical Manifestations of DIC

Cardiovascular
- Decreased pulses
- Decreased capillary filling time
- Tachycardia
- Venous distention

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

Assessment
Clinical Manifestations of DIC

Genitourinary
- Hematuria
- Oliguria

(consumption coagulopathy)

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

Assessment
Clinical Manifestations of DIC

Nervous System
- Vision changes
- Dizziness
- Headache
- Irritability
- Anxiety
- Confusion
- Seizures

(consumption coagulopathy)

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

Assessment
Clinical Manifestations of DIC

Musculoskeletal
- Joint pain
- Bone pain
- Weakness

(consumption coagulopathy)

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Diagnostic and laboratory tests

- Prothrombin time- prolonged
- Partial thromboplastin time prolonged
- Thrombin time prolonged
- Fibrinogen decreased
- Platelets- decreased
- *Fibrin split (degradation) products- elevated
- Factor assays (factors V, VII, VIII, X, XIII)reduced
- D-dimers- elevated

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Therapeutic management
- The priority of therapeutic management is to initiate
treatment of the underlying medical condition that
precipitated DIC.
- Life-threatening hemorrhage accomplished by
administering specific blood components based on the
identified deficiency: Platelets for thrombocytopenia;
cryoprecipitate to replace fibrinogen, and factors V and
VII; and fresh frozen plasma to replace all clotting
factors except platelets.
- Use of Heparin or Antithrombin III (AT-III) to control
intravascular clotting. Their use is controversial.

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

High priority nursing diagnosis:

- Impaired gas exchange;
- Altered tissue perfusion;
- Risk for fluid volume deficit;
- Pain;
- Decreased cardiac output

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

Planning and implementation

(consumption coagulopathy)

- Assess client carefully for evidence of bleeding and

altered tissue oxygenation
- Institute thrombocytopenic precautions
- Monitor intake and output hourly.
- Administer blood products as indicated by the health
care provider.
- *Monitor for signs of complications such as renal
failure, pulmonary embolism, cerebrovascular accident,
and acute respiratory distress syndrome
- Monitor effectiveness of therapy and pharmacologic
interventions including
- Provide emotional support to client and family.

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Medication therapy
- Heparin and Antithrombin III
- Epsilon aminocaproic acid (Amicar) to inhibit
fibrinolysis
- Blood products (FFP, platelets, and
cryoprecipitate).

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Client education
- Regarding the syndrome and explain
treatments and interventions
- To report symptoms of complications
including abdominal pain, headache, visual
disturbances, and pain.
- Thrombocytopenic precautions

Module 3

Hematology
Common Disorders of the hematologic system
H . Disseminated intravascular coagulation

(consumption coagulopathy)

Evaluation
- The clients hemodynamic status is
maintained.
- Peripheral pulses remain intact
- Skin remains intact.

Module 3

Hematology
Common Disorders of the hematologic system

I . Neutropenia
Description
Refers to a decrease (less than 2000/mm3) in the
neutrophil count either as a result of decreased
production or increased destruction. The
neutrophil plays a major role in phagocytosis of
disease-producing microorganisms.
Consequently, a decrease in their numbers
increases the individuals risk for infection.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

Etiology and pathophysiology

- Neutropenia is not a disease but a
syndrome.
- May occur as a primary hematologic
disorder but may also be caused by drugs,
autoimmune disorders, infections, and
other medical conditions such as severe
sepsis and nutritional deficiencies.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

Etiology and pathophysiology

- If the leukocyte count is decreased, or if
immature white blood cells predominate in the
circulation, the normal phagocytic function of
these cells is impaired.
- Neutrophils constitute about 70% of the
total circulating white blood cells. Normally,
the neutrophil count is above 2000/mm 3.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

Assessment
- There are no real symptoms associated
with neutropenia

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

Diagnostic and laboratory tests

- Neutrophil count less than 1000 to 1500.
- Bone marrow examination to examine
cell morphology helps distinguish the
etiologic factor causing the neutropenia.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

Therapeutic management
- If the etiology of neutropenia is drug-induced,
discontinuation of the medication is indicated.
- Corticosteroids are used if the etiology is
immunologic
- If the etiology is decreased production, growth
factors (granulocyte/macrophage colony
stimulating factor or GM-CSF) may be used.
- If client develops a fever, identification and
treatment of the infection is instituted

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

High priority nursing diagnosis

- Infection, risk for;
- Knowledge deficit
- Anxiety.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia
Planning and implementation:

- Monitor for signs of infection. Monitor temperature elevations

- Obtain cultures suspected as sites of infection.
- Administer antibiotics as prescribed and evaluate their
effectiveness.
- Administer medications that stimulate the production of
neutrophils.
- Enforce strict hand washing by all individuals in
contact with the client.
- Institute reverse isolation. Use private room with
HEPA filtration if possible.
- Avoid invasive procedures.
- Fresh flowers and fruits should not be permitted in the
clients room.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

Medication therapy
a. Growth factors such as G-SF
(Neupogen) or granulocyte/macrophagecolony-stimulating factor (Leukine) is
given to increase the neutrophil count.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

*Client education
- Teach client and family to report signs of fever
- Teach the client and individuals who come in
contact with the client about strict hand
washing and reverse isolation procedure.
- Teach client methods to maintain good
personal hygiene.
- Explain to the client and family about the
condition and the rationale of therapeutic
interventions.

Module 3

Hematology
Common Disorders of the hematologic system
I . Neutropenia

Evaluation
- The absolute neutrophil count normalizes.
- The client is free of infection.
- The client and family verbalize methods of
limiting exposure to pathogens.

Module 3

Hematology
Common Disorders of the hematologic system

J . Leukemia
Description
A malignant disorder of the blood-forming
tissues of the bone marrow, spleen, and
lymph system characterized by
unregulated proliferation of WBCs and
their precursors.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

Etiology and pathophysiology

- The type of WBC affected (granulocyte,
lymphocyte, monocyte) and the duration of the
disease (acute or chronic) is the basis of the
classification of the different types of leukemia.
*If the majority of the leukemia cells are
primitive, the leukemia is classified as acute; if
the leukemic cells are mostly mature (well
differentiated), the leukemia is classified as
chronic.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
Acute lymphocytic/lymphoblastic leukemia (ALL)
- Peak incidence at 2 to 4 years of age.
- Immature granulocytes proliferate and
accumulate in the marrow

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
Chronic lymphocytic leukemia (CLL)
- More common in men and mainly
between the ages of 50 and 70.
- Abnormal and incompetent lymphocytes
proliferate and accumulate in the lymph
nodes and spreads to other lymphatic
tissues and the spleen. Most of the
circulating cells are mature lymphocytes.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
Acute myelogenous/myelocytic leukemia (AML)
- All age groups are affected with a peak
incidence at age 60.
- There is uncontrolled proliferation of
myeloblasts, which are the precursors of
granulocytes. They accumulate in the
bone marrow.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
Chronic myelogenous leukemia (CML)
- Uncommon in people under 20 years of age.
The incidence rises with age.
- There is uncontrolled proliferation of
granulocyte resulting in increased circulating
blast (immature) cells.
- The marrow expands into long bones
because of this proliferation and also extends
into the liver and spleen.
- In most cases the Philadelphia chromosome,
a characteristic chromosomal abnormality, is
present.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

- Abnormal cells can continue to multiply,

infiltrate, and damage the bone marrow, spleen,
lymph nodes, liver, kidneys, lungs, gonads,
skin, and central nervous system (CNS)
- The bone marrow becomes functionally
incompetent with resulting bone marrow
suppression

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
- Acute leukemia has a rapid onset, progresses rapidly,
with a short clinical course; left untreated, death will result
in days or months. The symptoms of acute leukemia relate
to a depressed bone marrow, infiltration of leukemic cells
into other organ systems, and hypermetabolism of
leukemia cells.
- *Chronic leukemia has a more insidious onset with a more
prolonged clinical course. Clients with the chronic form of
leukemia are usually asymptomatic early in the disease. The
life expectancy may be more than five years. Symptoms of
chronic leukemia relate to hypermetabolism of leukemia cells
infiltrating other organ systems. The cells in this type of
leukemia are more mature and function more effectively.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
Clinical manifestations
- Fever
- Night sweats
- Bleeding
- Ecchymoses
- Lymphadenopathy
- Weakness
- Fatigue
- Pruritic vesicular lesions
- Anorexia
- Weight loss
- Shortness of breath

- Decreased activity tolerance

- Bone or joint pain
- Visual disturbances
- Gingival bleeding
- Epistaxis
- Pallor
- Splenomegaly
- Hepatomegaly

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

Diagnostic and laboratory tests

- Increased WBC (in CLL and CML)
- A normal, decreased or increased WBC (in ALL
and AML).
- *Decreased reaction to skin sensitivity tests
(anergy).
- Bone marrow tests reveal excessive blast cells in
AML
- Philadelphia chromosome found in 90% to 95% in
clients with CML. BCR/ABL gene is present in
virtually all clients with CML.Bone marrow biopsy
and aspirate is the definitive diagnostic test.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

Therapeutic management
- Induction of remission with chemotherapy
and radiation therapy.
- Bone marrow and stem cell
transplantation.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

High priority nursing diagnosis

- Risk for infection
- Risk for bleeding
- Altered nutrition, less than body
requirements
- Fatigue
- Activity intolerance
- Pain
- Grieving, anticipatory

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

Planning and implementation

- Review and institute the care of a client receiving
chemotherapy
- Review and implement the nursing care of a client
undergoing radiation therapy
- Assist in bone marrow biopsy. Apply pressure on
the site for five minutes or until bleeding stops.
Frequently assess the site for signs of bleeding up
to four hours after the procedure.
- Institute neutropenic and bleeding precautions
- Plan activities to prevent fatigue. Provide measures
for uninterrupted rest and sleep.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
Planning and implementation
- Provide for diversionary activities.
- Maintain good nutrition. Enlist the
assistance of a dietician in maximizing and
meeting the nutritional needs of the client.
- Assist the client in maintaining good
personal hygiene. Measures to promote
oral hygiene should be instituted.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia
Planning and implementation
- Refer client and family to appropriate agencies
such as Meals on Wheels, American Cancer Society,
and the Leukemia Society.
- Provide emotional support to the client and family.
Refer to appropriate agency, organization, or
professional for counseling and support.
- Administer drugs that are prescribed and monitor
for side effects.
- Monitor laboratory results to evaluate
effectiveness of interventions and therapy
- Prepare the client for bone marrow transplantation
if this is included in the treatment plan.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

Medication therapy
Chemotherapeutic drugs include:
- alkylating agents (Busulfan [Myleran]),
anthracyclines ( Doxorubicin [Adriamycin]),
- antimetabolites (Fludarabine [Fludara]),
- corticosteroid (Prednisone )
- plant alkaloids (Vincristine [Oncovin])
- others.

Module 3

Hematology
Common Disorders of the hematologic system
J . Leukemia

Evaluation
- The client has no infection.
- The client has no bleeding episodes.
- Client reports adequate pain control.
- Client tolerates activities of daily living.

Module 3

Hematology
Common Disorders of the hematologic system

K . Malignant lymphomas
Description
Lymphoma is a group of
malignant neoplasms that affects
the lymphatic system resulting in
the proliferation of lymphocytes.
Lymphomas can be classified as
- Hodgkins diease
- non-Hodgkins lymphoma.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Etiology and pathophysiology

Hodgkins Disease

- More common in men and has two peaks ; 15-35 years

of age and 55 to 75 years of age. Incidence is higher in
whites than in African Americans
- Cause unknown
- Several factors have been identified to contribute :
infection with the Epstein-Bar virus (EBV), familial
pattern, and exposure to toxins
- *Hodgkins disease is characterized by the presence of
Reed-Sternberg cell
- The tumor originates in a lymph node (in majority of
cases from the cervical nodes) and infiltrates the spleen,
lungs, and liver

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Etiology and pathophysiology

Non-Hodgkins Lymphoma

- Most common form of lymphoma. Affects usually

adults from 50 to 70 years old. It is more common
in men than women and in whites
- no known cause but is linked to viral infections,
immune disorders, genetic abnormalities, exposure
to chemicals, and infection with Helicobacter pylori
- has a similar pathophysiology to Hodgkins
disease although Reed-Sternberg cells are
absent and the method of lymph node infiltration is
different.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Etiology and pathophysiology

Non-Hodgkins Lymphoma

- In majority of cases the disease involves

malignant B cells
- lymphoma usually originates outside the
lymph nodes. The lymphoid tissues
involved become infiltrated with malignant
cells. The cells that make up the lymphoid
tissue become abnormal and crowd out
normal cells.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Clinical manifestations
Hodgkins Disease

- Usually begins with a firm and painless enlargement of

one or more lymph nodes on one side of the neck.
- Fatigue
- Weakness
- Anorexia
- Dysphagia
- Dyspnea
- PruritusDevelopment of severe but brief pain at the site
of Hodgkins after ingestion of alcohol.
- Cough
- Jaundice
- Abdominal pain
- Bone pain

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Clinical manifestations
Hodgkins Disease
B symptoms
- fever without chills
- night sweats
- unintentional 10% weight loss.
- Enlarged lymph nodes, liver, and
spleen

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Clinical manifestations
Non-Hodgkins Lymphoma

- Painless lymph node enlargement

- B symptoms (previous slide)
- Abdominal pain,
- Nausea
- Vomiting
- Hematuria
- Peripheral neuropathy
- cranial nerve palsies
- headaches
- visual disturbances
- changes in mental status and seizures
- Shortness of breath, cough, and chest pain.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Diagnostic and laboratory tests

Hodgkins Disease

- Normocytic, normochromic anemia

- Neutrophilia, monocytophilia, and lymphopenia
- *Presence of Reed-Strenberg cells in excisional
bone biopsy
- Mediastinal lymphadenopathy revelaed by chest
x-ray, CT scan, and radioisotope studies.
- Mediastinal mass and pulmonary infiltrates may
be seen on chest x-ray
- Absent or decreased response to skin sensitivity
testing known as anergy.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Diagnostic and laboratory tests

Non-Hodgkins lymphoma

- Lymphocytopenia
- X-ray may reveal pulmonary infiltrates.
- Lymph node biopsy helps to identify the
cell type and pattern

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Therapeutic management
Hodgkins Disease

- Lymphangiography is used to evaluate abdominal nodes.

- Staging laparotmoy is performed to obtain specimen of
retroperitoneal lymph nodes and to remove the spleen.
- Stage I indicates involvement of a single lymph node region ;
Stage IV (for Hodgkins disease only) indicates diffuse or
disseminated involvement of 1 + extralymphatic organs, with or
without lymph node involvement (liver, lung, marrow, skin).
- Radiation therapy for stages IA, IB. IIA, and IIB.
- Combination chemotherapy for stages III, IV and all B stages.
- Combination radiation and chemotherpay for stages IA and IB.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Therapeutic management
Non-Hodgkins lymphoma

- Staging of the disease is undertaken. This is based on data

obtained from CT scans and bone marrow bipsies
- Combination chemotherapy
- Radiation alone or in combination with chemotherapy for
stage I and II.
- Biologic therapy with alpha interferon, interleukin-2, and
tumor necrosis factor.
- Administration of rituximab (Rituxan), a monoclonqal
antibody against the CD20 of malignant B lymphocytes, which
causes cell lysis and death

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

High priority nursing diagnosis

- Risk for infection
- Activity intolerance
- Protection, altered
- Fatigue
- Altered nutrition, less than body
requirements
- Body image disturbance
- Hoplessness.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Planning and implementation

- Institue nursing interventions for clients on

chemotherapy or radiation therapy.
- Assist in balancing activity with periods of rest
- Provide and assist in maintaining good nutritional state
- Provide measures to diminish the discomfort
associated with pruritus
- Provide interventions to enable client to deal with
body image changes such as alopecia, weight loss,
and sterility.
- Refer client and family to appropriate agencies for
support such as the American Cancer Society.
- Plan interventions for the prevention of infection.

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Medication therapy :
- Chemotherapeutic agents
- Biologic therapy agents

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Client education
- nature of the disease, the course of
therapy and associated interventions.
- medications prescribed, preacautions,
and side effects.
- symptoms necessitating immediate
medical intervention such as the
occurrence of bleeding, infection, or fever

Module 3

Hematology
Common Disorders of the hematologic system
K . Malignant lymphomas

Evaluation
- The client does not develop complications
of bleeding or infection.
- The client regains normal weight
- The client verbalizes absence of pain
- The client and family verbalizes
understanding of reasons of on-going
treatment and interventions

Module 3

Hematology
Common Disorders of the hematologic system

Test
1. A client who has undergone a small
bowel resection of the ileum is most
likely to develop which type of anemia?
1. Iron deficiency anemia
2. Vitamin B12 deficiency anemia
3. Anemia of chronic disease
4. Aplastic anemia

Module 3

Hematology
Common Disorders of the hematologic system

Test
Answer: 2
Rationale: Resection of the distal ileum results
in the impaired absorption of vitamin B12. The
other cause of Vitamin B12 deficiency is due to
the loss of intrinsic factor secreting surface that
are normally secreted by parietal cells.

Module 3

Hematology
Common Disorders of the hematologic system

Test
2. A client is scheduled for a test to
determine if pernicious anemia is
present. Which of the following tests
should the nurse schedule the client for?
1. Serum folate level
2. Schilling test
3. Serum iron and total iron- binding
capacity (TIBC)
4. Bone marrow aspiration

Module 3

Hematology
Common Disorders of the hematologic system

Test
Answer: 2
Rationale: Schilling test involves the
administration of radioactive Vitamin B12.
Increased absorption of vitamin B12 when
intrinsic factor is given parenterally is indicative
of pernicious anemia

Module 3

Hematology
Common Disorders of the hematologic system

Test
3. The nurse is assessing a group of clients and
identifies which of the following as being at high
risk for the development of folic acid deficiency
anemia?
1. Obese individuals
2. Alcoholics
3. Young adults
4. Athletes

Module 3

Hematology
Common Disorders of the hematologic system

Test
Answer: 2
Rationale: Individuals who are chronically
undernourished including the elderly, alcoholics,
substance abusers, and those with high metabolic
requirements and on total parenteral nutrition are at
risk for folic acid deficiency anemia. Alcoholics are
particularly at risk because alcohol interferes with
folate metabolism.

Module 3

Hematology
Common Disorders of the hematologic system

Test
4. Which of the following questions during
the data-gathering phase is important for the
nurse to ask in a client suspected of having a
nutritional anemia?
1. Do you have a sore tongue?
2. How is the consistency of your stools been?
3. Do you experience any tingling or numbness?
4. Have you had blood transfusions in the
past?

Module 3

Hematology
Common Disorders of the hematologic system

Test
Answer: 3
Rationale: The differentiating symptom of Vitamin
B12 and Folic acid deficiency anemia is the
absence of neurologic symptoms such as
numbness and altered proprioception in folic acid
deficiency anemia. The gastrointestinal symptoms
of cheilosis, glossitis, and diarrhea are present in
both forms of nutritional anemia although usually
more severe in folic acid deficiency anemia

Module 3

Hematology
Common Disorders of the hematologic system

Test

5. A couple seeks genetic counseling for sickle

cell. Both have sickle cell traits. The nurse
understands that the chances of the couples
offspring developing sickle cell disease with
each pregnancy is:
1. None of the offspring will develop sickle cell disease
2. Twenty five percent of their offspring will develop sickle
cell disease
3. Fifty percent of their offspring will develop sickle cell
disease.
4. All their children will have sickle cell traits but none will
have the disease

Module 3

Hematology
Common Disorders of the hematologic system

Test
Answer: 2
Rationale: Sickle cell disease is an autosomal
recessive genetic disorder where the individual is
homozygous for the abnormal hemoglobin. If both
parents have sickle cell traits, there is a 25%
chance that each pregnancy will produce a child
with the disease