Hereditary spherocytosis should be suspected if a number of findings are identified. The diagnosis requires genetic testing to identify the precise mutation.
Reasons to suspect that a patient has hereditary spherocytosis:
(1) family history of hereditary spherocytosis
(2) family history of hereditary anemia
(3) chronic unexplained anemia
(4) presence of spherocytes in the peripheral blood smear
(5) evidence of a antiglobulin-negative hemolytic anemia with variable jaundice
(6) splenomegaly
(7) onset of cholelithiasis in an adolescent or young adult
(8) aplastic or hemolytic crisis triggered by a viral infection
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