Wednesday, September 23, 2015

Spleen congenital haemolytic anaemia


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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