Abstract
haemolytic anaemia, splenomegaly and jaundice, admitted in Chettinad hospital and research institute during one year period from January 2019 to January 2020. Surgical intervention is indicated for selected patients with hereditary spherocytosis with haemolytic anaemia and jaundice to abate the hemolytic process after correction of anaemia with blood transfusion. Pigmented gallstones are seen in more than 50% cases for which incidence increases with severity of hemolysis and with age. Complications include aplastic anemia (most common after parvovirus B19 infection), haemolytic crisis during inter-current infection, megaloblastic crisis – during folic acid deficiency, cardiomyopathy, hematological malignancies.
Highlights
Hereditary spherocytosis (HS) is a rare type of congenital haemolytic anaemia that occurs world wide
The use of partial splenectomy aims to retain splenic immunologic function, while at the same time to decrease the rate of hemolysis
The longterm outcomes of patients with total or subtotal splenectomy for congenital hemolytic anemia, still remain unclear, but the majority of the studies showed a qualitative resolution of anemia and reduction of transfusion rate
Summary
Hereditary spherocytosis (HS) is a rare type of congenital haemolytic anaemia that occurs world wide. Age at diagnosis of HS is often between 3 – 7 years but can occur in infancy with severe disease or into adult hood with mild disease. 2 that link the bilayer of red cells to the membrane skeleton Loss of this protein causes red blood cells to lack their characteristic biconcave shape. These proteins are more susceptible to trapping and destruction by the spleen resulting in haemolysis, splenomegaly and cholelithiasis. The passage of these RBCs into sinusoids is difficult and gets phagocytosed resulting in extravascular haemolysis. Complications include aplastic anemia (most common after parvovirus B19 infection), haemolytic crisis during inter-current infection, megaloblastic crisis – during folic acid deficiency, cardiomyopathy, hematological malignancies
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