44.

Nursing Care of the Child with a Hematologic Disorder

Terms
1. Agranulocytes - white blood cells without granules in the cell cytoplasm
2. Allogeneic Transplantation - transfer of body tissues from an immune compatible donor
3. Aplastic Anemia - depression of hematopoietic activity in bone marrow affecting all blood cells
4. Autologous transplantation - transplantation using the person’s own previously removed tissue
5. Blood dyscrasias - hematologic disorders
6. Blood plasma - liquid portion containing proteins, hormones, enzymes, and electrolytes
7. Direct bilirubin - water-soluble end breakdown product of heme that is combined and excreted in bile
8. Erythroblasts - large, nucleated early red blood cells
9. Erythrocytes - red blood cells
10. Erythropoietin -a hormone produced by the kidneys that stimulates red blood cells development
11. Granulocytes - white blood cells with granules in the cell cytoplasm
12. Heinz bodies - oddly shaped particles in red blood cells
13. Hemochromatosis - deposition of iron in body tissues from destruction of red blood cells
14. Hemoglobin - a complex protein that is the oxygen-carrying component of red blood cells
15. Hemolysis - destruction of red blood cells
16. Hemosiderosis - deposition of iron in body tissues
17. Hypodermoclysis - subcutaneous infusion
18. Leukocytes - white blood cells
19. Leukopenia - a decrease in the number of white blood cells
20. Megakaryocytes - immature thrombocytes
21. Normoblasts - a developmental stage of RBC after erythroblasts
22. Pancytopenia - reduction of all blood cell components
23. Petechiae - pinpoint, macular, purplish-red spots caused by intradermal or submucous hemorrhage
24. Plethora - marked reddened appearance of the skin
25. Poikilocytic - irregular in shape
26. Polycythemia - an increase in the number of red blood cells that results as a compensatory response to insufficient oxygenation of
the blood
27. Priapism - persistent, painful erection
28. Purpura - hemorrhagic rash or small hemorrhages occurring in the superficial layer of skin
29. Reticulocytes - a developmental stage of red blood cells prior to the formation of mature RBCs
30. Sickle cell crisis - term used to denote a sudden, severe onset of sickling with sickle-cell anemia
31. Sickle cell trait - the existence of hemoglobin and abnormal hemoglobin, a disease carrier
32. Synergetneic transplantation - transplantation between a donor and recipient who are generally identical
33. Thrombocytes - platelets
34. Thrombocytopenia - decreased platelet count

Components of the Blood

- Plasma - 55%
- Formed elements - 45%
A. Erythrocytes
▪hemoglobin as the primary component
▪oxyhemoglobin
▪Carbaminohemoglobin
B. Leukocytes
C. Thrombocytes

Plasma Proteins (fibrinogens, albumin, globulins)

Albumin - important protein in blood
-promotes “oncotic pressure” which is a pulling force of H2O
*if less albumin, there’s leaking of water that would cause edema
Fibrinogens - clotting process
Globulins - IgA, IgB, IgE, IgM, IgG

Formed Elements
A. Thrombocytes (platelets) - coagulation - causes blood clot (normal level of platelets 150,000-450,000 mm3)
Dengue fever - decrease platelet; caused by virus that attacks platelets
 Clotting Process

Extrinsic Pathway (wound) Intrinsic Pathway

Tissue Trauma Damaged in endothelial

cells/platelets

Release of tissue Factor

Activates Factors XII
Ca²⁺
Activates Clotting Factor X
Activates Factors X

Ca²⁺ Formation of Prothrombin II

Ca²⁺

Converts Prothrombin II to thrombin

Ca²⁺

Converts Fibrinogen I Activates Factor XII

Loose Fibrin threads Strengthens and stabilizes the fibrin thread

Study clot
B. Leukocytes (WBC)
A. Granular Leukocytes -
1. Basophils - anti-inflammatory
2. Eosinophils - activates when there is helminths (parasites) and allergic reactions
3. Neutrophils- photocyte
B. Agranular Leukocytes -
1. Monocytes - big phagocytes
2. Lymphocytes - T-Lymphocytes & B Lymphocytes
*Increased WBC indicates infection

C.Erythocytes (RBC) - Produced in bone marrow

-120 days lifespan
-Transport oxygen
Hemoglobin - carries oxygen
Hematocrit - percentage of erythocytes in the blood

*If hematocrit is low (30%): Decreased RBC (hemorrhage or anemia);

What prompts the bone marrow to produce RBC?

=Kidney monitors the Oxygen and production of O2 and releases special enzyme called “Renal Hematopoietic Factor” that would
travel and reach the liver and convert Erythropoietinogen and convert again to Erythropoietin which is delivered to bone marrow and
would induce erythropoiesis.

What makes up RBC?

1. Iron - component of hemoglobin that causes attraction of Oxygen
Hemoglobin - Heme - iron part
Globin - Protein
2. Vitamin B12
3. Amino Acids
4. Cobalt
5. Copper
6. Globin
7. Folic Acid

5 Reasons why RBC is a specialize cell for O2 transportation

1. Biconcave shape - allows greater surface for RBC to accommodate more O2
2. Flexible
3. Has no nucleus
4. Has no mitochondria
5. Most abundant cell in the blood and presence of hemoglobin

Hematologic Disorders
Condition Signs and Symptoms Management
Hemophilia -prolonged bleeding from any - Control bleeding
-defects in clotting mechanism of blood : two wound -Prevention of bleeding with use of factor
most common deficiencies are: -Bleeding into the joints replacement
1. Factor VIII, classic hemophilia (hemarthrosis) resulting to A. Drugs that replace deficient coagulation
2. Factor IX, Christmas disease pain, deformity and retarded Factors
growth 1. Antihemophilic Factor - obtained from
- hereditary influence: X-linked gene -anemia human sources: provides concentrated
classically occurring in males, and females as -epistaxis Factor VIII
carrier -Intracranial hemorrhage 2. Antihemphilic plasma
-Platelet count - normal 3. Factor IX complex contains factors II,
-Prolonged coagulation time VII, X (concentrated)
-Increased PTT B. Adjunctive Measures
1. Episilon-aminocaproic acid (Amicar): inhibits
the enzyme that destroys formed fibrin and
increase fibrinogen activity in clot formation
 2. Fibrinogen: maintains plasma fibrinogen levels
required for clotting materials
3. Thrombin : supplies physiologic levels of
natural material at superficial bleeding sites to
control bleeding
Nursing care:
1. Immobilize of the affected joints
2. Compression of the bleeding site
3. Elevation of the body part
4. Application of cold compresses
5. Select safe toys: prevent trauma
6. Avoid use of aspirin or ibuprofen
7. Provide counseling since disease is genetic
8. Treat the child as normally as possible, avoid
overprotectiveness and overpermissiveness
Sickle Cell Anemia - Colic - 1st sign in infants *Prevent Crisis:
-Autosomal disorder affecting hemoglobin (Severe pain in joints, back, 1. Avoid infection, DHN, and othe rconditions
-defective hemoglobin causes RBC to become extremities) causing strain on body, which precipitates a crisis:
sickle shaped and clump together under -Difficulty concentrating urine prophylactic use of flu vaccines
reduced oxygen tension -Frequent infections 2. Avoid hypoxia, treat respiratory tract infections
-chronic, severe, hemolytic disease _Delayed Growth and STAT
development 3. Avoid DHN - causes a rapid thrombin formation
Sickle Cell Crisis: -Hb 6-9 g/dl 4. Daily fluid intake should be calculated
* Vaso-occlusive crisis: most common and - decreased ESR, increased according to body wt. (130-200 ml per kg). During
only painful type: results from sickled cells WBC crisis, fluid needs to be increased especially if the
obstructing blood vessels causing occlusion, -Vaso-occlusive crisis: child is febrile
ischemia, and potential necrosis *fever, acute abd’l pain
(visceral hypoxia) *During crisis:
*Splenic Sequestration crisis: results form the *hand-foot syndrome 1. Adequate hydration (may need IV therapy)
spleen pooling large quantities of blood, which (dactylitis - symmetrical 2. Proper positioning, careful handling
causes a precipitous drop in blood pressure and painful soft tissues swelling of 3. Exercise is tolerated (immobility promotes
ultimately shock: acute episodes occurs most hands and feet in the absence of thrombus formation and respiratory problems)
commonly in children between 8 months - 5 trauma) 4. Adequate ventilation
years of age, can result in death from anemia *arthralgia without an 5. Control of pain: use of narcotic analgesics
and cardiovacular collapse: Chronic exacerbation of anemia 6. Blood transfusions for severe anemia
manifestation is termed functional asplenia
-Splenic Sequestration Crisis *Genetic counseling:
* Aplastic Crisis: diminished RBC production: * preicipitous decrease in BP 1. Disorder mostly occur in blacks: can be found
may be triggered by a viral or other infection *Shock in Mediterranean people
2. Screen young children for the d/o since clinical
*Hyperhemolytic crisis: Increased rate of RBC Aplastic crisis: manifestations usually do not appear before 6
destruction *Profound anemia months of age
3. If both parents are carriers, each pregnancy has
Hyperhemplotic crisis: 25% chance of producing a child with the disease
-jaundice, anemia
-reticulocytosis
Leukemia Acute Leukemia * Acute Leukemia
* Acute Leukemia -sudden onset of high fever -Systemic chemotherapy
-is a malignant proliferation of white blood -thrombocytopenia and -Bone marrow transplant may be possible
cell precursors (blasts) in bone marrow or abnormal bleeding such as -Antibiotic, antifungal and antiviral drugs
lymph tissue and their accumulation in nosebleeds, petechiae, easy -Transfusion may also be given of platelets to
peripheral blood, bone marrow and body bruising after minor trauma and prevent bleeding and red blood cells to prevent
tissues. prolonged menstruation anemia

*Leukemia, Chronic Granulocytic Leukemia, Chronic Leukemia, Chronic Granulytic

Characterized by abnormal overgrowth of Granulycytic -Aspirin to prevent stroke
granulocytic precursors in bone marrow and -Anemia (fatigue, weakness, -Local splenic radiation or splenectomy to increase
peripheral blood and body tissues. decreased exercise tolerance, platelet count
 pallor, dyspnea, tachycardia -Leukapheresis or selective leukocyte removal
-thrombocytopenia -Allopurinol to prevent secondary hyperuricemia
-hepatosplenomegaly with or colchicine to prevent gout
abdominal discomfort and pain
in splenic infarction
*Leukemia, Chronic Lymphocytic Leukemia, Chronic Leukemia, Chronic Lymphocytic
Is marked by an uncontrollable spread of Lymphocytic -Systemic chemotherapy includes alkylating
abnormal, small lymphocytes in lymphoid Early Stage:fatigue, malaise, agents, usually chlorambucil or cyclophosphamide
tissue, blood, and bone marrow. fever and nodal enlargement and sometimes steroid when autoimmune
hemolytic anemia or thrombocytopenia occurs.
Advance stage:weight loss, -Local radiation treatment
liver or spleen enlargement,
bone tenderness, and edema
from lymph node obstruction
-bone marrow involvement
may lead to anemia, pallor,
weakness bleeding and
infection