Cholangitis associated with paroxysmal nocturnal hemoglobinuria: Another instance of ischemic cholangiopathy?
1995; Elsevier BV; Volume: 109; Issue: 4 Linguagem: Inglês
10.1016/0016-5085(95)90598-7
ISSN1528-0012
AutoresD. Lê Thi Huong, Dominique Valla, Dominique Franco, B Wechsler, Aimery de Gramont, Andre Auperin, P Godeau,
Tópico(s)Renal Diseases and Glomerulopathies
ResumoIschemia is increasingly recognized as a cause of cholangiopathy. The aim of this study was to report a case of association of paroxysmal nocturnal hemoglobinuria with abrupt-onset cholangiopathy. Anomalies resembling sclerosing cholangitis were documented in a patient suffering from recurrent biliary pain. None of the conditions that have been associated with primary sclerosing cholangitis or other forms of cholangiopathy was present, but shortly thereafter, paroxysmal nocturnal hemoglobinuria occurred. Hepatic vein thrombosis later complicated the course of the disease. Because the fortuitous coincidence of these uncommon conditions is unlikely, this case indicates that paroxysmal nocturnal hemoglobinuria is a cause of ischemic cholangiopathy. Other thrombogenic conditions may also be implicated in some instances of apparently idiopathic cholangiopathy.
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