A patient with Wilson's disease may present with a hemolytic anemia.
Features of the anemia:
(1) Coombs-negative hemolytic anemia.
(2) Hemolytic crises can occur, sometimes in conjunction with fulminant hepatic necrosis.
Pathogenesis:
(1) free radicals
(2) ceramide and acid sphingomyelinase affecting red blood cell membranes
The diagnosis is often made after other features of Wilson's disease are identified.
Differential diagnosis:
(1) pancytopenia due to copper deficiency from excess chelation therapy
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Specialty: Hematology Oncology, Clinical Laboratory
