Heriditary spherocytosis

Autosomal dominant
Charctrsd by defi of spectrin, a red cell membrane protein. This results in sphericl erytrocytes whch r more susceptibl 2 osmotic lysis

-Strong family H/O of anaemia, jaundice, splenomegaly & cholelithiasis
-25% of d patients hav mild compensatd haemolysis
-In chldrn growth retard due 2 hemolysis & bone changes due 2 marow hypertrophy
-In adults, anaemia, intermitent jaundice & modrat splenomegly

#Chronic leg ulcers
#Hemolytic crisis
#Aplastic crisis due 2 parvovirus infection

Demo of a hemolytic state
Demo of spherocytes in bld film
Increasd osmotic fragility
-ve Coomb's test
Demo of same disordr in other family membrs

Splenectomy is Rx of choice. It shud nt b done until d age of 6 yrs. Splenectomy shud b preceded by pneumococcal & H. influenzae imunization & folowd by liflong penicilin prophylaxis PenV 500mg BID

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