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April 1979

Hemolysis, Coagulation Defects, and Fulminant Hepatic Failure as a Presentation of Wilson's Disease

Author Affiliations

Departments of Pediatric Hematology and Laboratory Hematology Cleveland Clinic Foundation 9500 Euclid Ave Cleveland, OH 44106

Am J Dis Child. 1979;133(4):440-441. doi:10.1001/archpedi.1979.02130040094024

Fulminant hepatitis with acute hemolysis is a rare but well established presentation of Wilson's disease.1.2 Several case reports1.3 have suggested that coagulation disorders occur in conjunction with this clinical picture. We have recently cared for a patient with fulminant hepatic failure and acute hemolysis due to Wilson's disease in whom striking coagulation abnormalities were detected in the laboratory in the virtual absence of clinical hemorrhage.

Report of a Case.—This 17-year-old girl was transferred to the Cleveland Clinic for evaluation of episodic hemolytic anemia. Five months before her admission, she had been evaluated elsewhere for hemolytic anemia and jaundice. Hemoglobin level during that admission was 5.1 g/dL; reticulocyte count, 33%; SGOT, 85 IU/L (normal, 8 to 33 IU/L); and lactic dehydrogenase, 490 units/dL (normal, 100 to 225 units/dL). This episode subsided spontaneously. She had no evidence of autoimmune hemolysis or systemic lupus erythematosus. For two weeks prior to

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