Heamatology 3
Heamatology 3
Heamatology 3
COURSE: HEAMATOLOGY
ASSIGNMENT NO#: 2
a) Autoimmune cold and warm antibody types associated with the appearance of antibodies in
the organism against their own erythrocytes
Causes
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;
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.
Antibody produced by one individual reacts with red cells of another. This is seen in
Infections
• They may precipitate an acute haemolytic crisis e.g. in G6PD deficiency or cause
microangiopathic haemolytic anaemia (e.g. with meningococcal or pneumococcal
septicaemia).
Clostridium perfringens septicaemia can cause intravascular haemolysis with marked micro
spherocytosis
• 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
• 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
• Neutrophil dysfunction.
• Renal insufficiency.
• Dysphagia.
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.
• The autoantibody attaches to red cells mainly in the peripheral circulation where the
blood temperature is cooled.
• 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
to acute, severe isovolemic anemia. JAMA 1998; 279:217
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;
111:439
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