5. CONGENITIAL HAEMOLYTIC ANAEMIA
Hereditary spherocytosis is a relatively common genetic
linked
disorder characterized by :
*
Small dense, spheroid erythrocytes
*
Increased osmotic fragility because of a red cell
membrane
defect
*
These cells are impeded through the spleen and destroyed
Patients frequently
present with a family
history and past
history of attacks of jaundice in
childhood and in early life.
Besides anaemia the patients:
*
In acute crisis have accompanied fever and chills
*
In later life may have bilirubin gallstones
*
Show splenomegaly as a characteristic feature
*
Spherocytes can be demonstrated in the peripheral blood smear
*
Osmotic fragility of the R.B.C. is readily demonstrable
TREATMENT
Patients are benefitted by splenectomy and is
recommended after
the age of six.
Basic
cellular defect persists after splenectomy; but the cells
survive normally.
Cholecystectomy is
recommended when stones are present
in the
gallbladder
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