RESOLUTION OF BUDD-CHIARI SYNDROME FOLLOWING BONE-MARROW TRANSPLANTATION FOR PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA

Thrombosis of the hepatic veins (Budd-Chiari syndrome) is a life-threa tening thrombotic complication which can occur in patients with paroxy smal nocturnal haemoglobinuria (PNH). Despite aggressive medical and s urgical therapy, mortality from Budd-Chiari syndrome remains high. We report a boy with PNH who developed Budd-Chiari syndrome and underwent syngeneic bone marrow transplantation (BMT). Now, 3 years following B MT, he has had dramatic clinical and radiographic evidence of resoluti on of the thrombosis. We suggest that BMT for PNH can successfully cor rect life-threatening thrombosis in patients with PNH.