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Acute Intravascular Hemolysis and Acute Liver Failure Associated as a First Manifestation of Wilson's Disease

JACQUELINE ROCHE-SICOT, M.D.; and JEAN-PIERRE BENHAMOU, M.D.
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▸Requests for reprints should be addressed to Jean-Pierre Benhamou, M.D.; Hôpital Beaujon; 92110 Clichy, France.


Clichy, France


Ann Intern Med. 1977;86(3):301-303. doi:10.7326/0003-4819-86-3-301
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In three patients, the first manifestation of Wilson's disease was a syndrome in which acute intravascular hemolysis and acute liver failure were associated. This syndrome developed in three periods; the first, lasting 3 to 14 days, was characterized by fatigue, fever, and jaundice; the second, lasting 1 or 2 days, by severe acute intravascular hemolysis; and the third, lasting 2 to 6 days, by hepatic encephalopathy. All of the patients died from liver failure 7 to 21 days after the onset of the syndrome. The association of acute intravascular hemolysis and acute liver failure is a characteristic manifestation of Wilson's disease; it is rarely associated with other liver diseases. This association might result from hepatic cell necrosis due to accumulation of copper, the consequences being acute liver failure and destruction of erythrocytes by the large amounts of copper released from the necrotic hepatic cells to the plasma.

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