Heamatology 3

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SCHOOL OF BIOMEDICAL SCIENCES BSC

BIOMEDICAL SCIENCES YEAR IV

NAME: CHAMA KABASO

COMPUTER NO#: 201912010459

COURSE: HEAMATOLOGY

LECTURER: MR MODERN NTALASHA

ASSIGNMENT NO#: 2

TASK: Discuss acquired immune hemolytic aneamia.

DUE DATE; 5th September 2021


Haemolytic anemia is anaemia due to increased destruction of the red blood cells. Increased
destruction causes shortened lifespan of RBC’s and they live for less than 90 days. Hemolysis of
RBC’s is a result of abnormalities of the RBC’s. Lysis of the red blood cells leads to the release
of bilirubin into the plasma.

Acquired immune heamolytic anemia is a type of heamolytic anemia which is caused by


extrinsic factors. It is divided into two groups;

a) Autoimmune cold and warm antibody types associated with the appearance of antibodies in
the organism against their own erythrocytes

b) Alloimmune: hemolytic transfusion reactions, hemolytic disease of newborn.

Causes

 Drug associated acquired hemolytic anemia


Thrombotic thrombocytopenic purpura Meningococcal sepsis DIC (disseminated
intravascular coagulation)
 Infection e.g. malaria
 Chemical and physical agents e.g. drugs, burns, industrial chemicals
 Secondary acquired hemolytic anemia e.g. due to liver or renal disease

AUTOIMMUNE ACQUIRED HEAMOLYTIC ANEMIA;

Antibodies ( IgG) optimally react at 37 C. In this case of autoimmune hemolytic anemia, there is
production of antibodies in the organism against their own erythrocytes. The mechanism is by
disruption of immunological sensitivity to the organisms own erythrocytes. This can happen in
any age or sex and it is usually associated with immunodeficiency syndrome and may also be
drug induced. The red cells are destroyed extravasiculary.

Causes

 Idiopathic
 Secondary Infection eg SLE, Lymphomas, drugs(methyl dopamine)

Symptoms

I. Dyspnoea
II. Fatigue
III. Palpitations
IV. Patients may be mildly jaundiced due to increased bilirubin
V. In severe cases it may be fast developing anaemia with hepatitis.
VI. Sometimes patients have moderate splenomegaly and lymphadenopathy

Diagnosis;

 Normocytic and normochromic anemia


 Hb levels are lower than normal
 Always increased reticulocyte count common in all types of hemolytic anemia

Treatment

Treatment is usually with steroid hormones e.g. prednisolone. The first dose is 60mg/day for
adults. If there is no response, the daily dose is increased up to 200mg/day. If there is still no
response, the second step is splenectomy. If splenectomy is not effective, use
immunodepressants e.g. cyclophosphamine. This however cannot be used in HIV patients. In
severe cases, blood transfusion is done but compatible donors are very difficult to find due to
immunoglobulin sensitivity.

ALLOIMMUNE HEMOLYTIC ANEMIA

Antibody produced by one individual reacts with red cells of another. This is seen in

 Transfusion of ABO – incompatible blood.


 Haemolytic disease of the newborn.
 Allografts

Alloimmune hemolytic anemia A good example of alloimmune hemolytic anemia is transfusion


reaction of the hemolytic disease of the newborn which happens when the mother has 𝑅ℎ − and
father has 𝑅ℎ + 𝑏𝑙𝑜𝑜𝑑. Hypersplenism is a condition in which there is an excess in one of the
normal functions of the spleen and as a result it affects blood cells. Often, it is a complication of
splenomegaly. It occurs together with thrombocytopenia, leukopenia and anemia (this
combination is pancytopenia).

Infections

• Infections can cause haemolysis in a variety of ways:

• They may precipitate an acute haemolytic crisis e.g. in G6PD deficiency or cause
microangiopathic haemolytic anaemia (e.g. with meningococcal or pneumococcal
septicaemia).

• Malaria causes haemolysis by extravascular destruction of parasitized red blood cells as


well as by direct intravascular lysis.

• Blackwater fever is an acute intravascular haemolysis accompanied by acute renal failure


caused by Falciparum malaria.

Clostridium perfringens septicaemia can cause intravascular haemolysis with marked micro
spherocytosis

Drug - induced immune haemolytic anaemias.

• Drugs can cause immune haemolytic anaemias through three mechanisms being:

I. Antibody directed against a drug – red cell membrane complex (e.g. penicillin,
ampicillin). This only occurs with massive doses of the antibiotic.

II. Deposition of complement via a drug – protein (antigen) – antibody complex onto the red
cell surface (e.g. quinidine, rifampicin)

III. A true autoimmune haemolytic anaemia in which the role of the drug is unclear (e.g.
methyldopa).
Paroxysmal Nocturnal Haemoglobinuria

• Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder, which


arises following a somatic mutation in a multipotential stem cell.

• Nocturnal haemolysis was once thought to be associated with a slight drop in plasma pH
during sleep, but this is now known to be untrue.

Characteristics

• RBC’s are susceptible to the lytic action of complement.

• Chronic but episodic intravascular haemolysis.

• Occurs mainly during sleep.

• Moderately to severe panhypoplasia.

• Neutrophil dysfunction.

• Renal insufficiency.

• Dysphagia.

• Tendency to venous thrombosis.

Broadly, the red cells in cases of PNH can be divided into three groups:

1- PNH I red cells are normal with regard to their sensitivity to complement-mediated lysis.

2- PNH II red cells show a moderately increased sensitivity.

3- PNH III red cells show a markedly increased sensitivity

Cold autoimmune haemolytic anaemias

• The autoantibody attaches to red cells mainly in the peripheral circulation where the
blood temperature is cooled.

• The antibody is usually IgM and binds to red cells best at 4 ° C.

• IgM antibodies are highly efficient at fixing complement and both intravascular and
extravascular haemolysis occurs.
• It is clinically presented with features of haemolytic anaemias with acrocyanosis.

• Laboratory findings are similar to those of warm AIHA except that spherocytosis is less
marked.
REFERENCES
1. World Health Organization. Nutritional anaemias: Report of a WHO scientific group.
Geneva, Switzerland: World Health Organization; 1968
2. Weiskopf RB, Viele MK, Feiner J, et al. Human cardiovascular and metabolic response
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3. Cook JD, Skikne BS. Iron deficiency: definition and diagnosis. J Intern Med 1989;
226:349.
4. Bryant BJ, Yau YY, Arceo SM, et al. Ascertainment of iron deficiency and depletion in
blood donors through screening questions for pica and restless legs syndrome.
Transfusion 2013; 53:1637.
5. Spencer B. Blood donor iron status: are we bleeding them dry? Curr Opin Hematol 2013;
20:533.
6. Annibale B, Capurso G, Chistolini A, et al. Gastrointestinal causes of refractory iron
deficiency anemia in patients without gastrointestinal symptoms. Am J Med 2001;
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7. Bridges KR, Seligman PA. Disorders of iron metabolism. In: Blood: Principles &
Practice of Hematology, Handin RI, Lux SE, Stossel TP (Eds), 1995. p.ch.49
8. Fairbanks VF. Laboratory testing for iron status. Hosp Pract 1990; 26:17
9. Martin A, Thompson AA. Thalassemias. Pediatr Clin North Am 2013; 60:1383.
10. Forget BG. Thalassemia syndromes. In: Hematology: Basic Principles and Practice, 3rd
ed, Hoffman R, Benz EJ Jr, Shattil SJ, et al. (Eds), Churchill Livingston

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