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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