Resolution of Budd-Chiari syndrome following bone marrow transplantation for paroxysmal nocturnal haemoglobinuria.
Thrombosis of the hepatic veins (Budd-Chiari syndrome) is a life-threatening thrombotic complication which can occur in patients with paroxysmal nocturnal haemoglobinuria (PNH). Despite aggressive medical and surgical 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 BMT, he has had dramatic clinical and radiographic evidence of resolution of the thrombosis. We suggest that BMT for PNH can successfully correct life-threatening thrombosis in patients with PNH.
Graham
M L
ML
Department of pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
Rosse
W F
WF
Halperin
E C
EC
Miller
C R
CR
Ware
R E
RE
D016026:Bone Marrow Transplantation; D006502:Budd-Chiari Syndrome / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D002648:Child; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008099:Liver / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male