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06-06-2008, 02:30 PM
m Definition: Massive destruction of R.B.CS.
m Aetiology:
(I) Corpuscular abnormalities: "1ry" due to.
a) Membrane defect e.g. spherocytosis "commonest".
b) Hg. defects e.g. thalassaemia.
c) Enzyme defect e.g. Glucose 6 phosphat dehydrogenase def.
(II) Extracorpuscular causes: "2ry" due to:
Physical agents, drugs, poisons, passive antibody tr, hypersplenism.



PRIMARY HAEMOLYTIC ANAEMIA (SPHEROCYTOSIS)
m Definition:
Cong. defect in the membrane of R.B.C.S. leads to biconvex corpuscles which are more fragile (easily destructed) ® ­ haemolysis of R.B.C.S.
m Pathology:
1- ­ Haemolysis ® enlarged liver and spleen.
2- Exaggeration of normal bile pigment cycle ®
Increase in haembilirubin in blood.
Cholebilirubin formation is fixed as it is adjusted by the liver function but it reach its peak.®­ cholebilirubine in stool ®­stercobilin.
Increase urobilinogen (urine become dark if exposed to air for long time)
Ä Cholebilirubine secretion in urin not increased ® Normal colour of urine (Acholuric jaundice)
Ä Pigment stone formation
m Clinical Picture:
1- Pallor, jaundice "Lemon yellow colouration of skin".
2- Spleen and liver may be felt.
3- Haemolytic crisis may occur ® fever, severe anaemia and increase the depth of jaundice.
4- In long standing cases may give:
- Ch. leg ulceration which heal after splenectomy.
m Investigations:
1- R.B.CS. count ®anaemia.
2- Icterus index "Noraml up to 6 Units": increase not > 50u.
It is done by comparison bet. colour of the serum with standard soln.
3- Indirect V.D.R.
4- Fragility tests ® Fragile R.B.CS.
5- Ultrasonography ® stone in G.B. may be detected, enlarged liver and spleen.
m Treatment: Splenectomy ® releive both anaemia & jaundice.
SOURCE: DR. AYMAN SALEM'S BOOK
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