Severe hepatic veno-occlusive disease (VOD) which was successfully treated with supportive therapy, but subsequently developed late-recurrence

• Published in: Rinshō ketsueki Vol. 39; no. 2; p. 139
• Main Authors: Matsuoka, S, Okamoto, S, Ishida, A, Wakui, M, Watanabe, R, Moriki, T, Ikeda, Y, Hirabayashi, N
• Format: Journal Article
• Language: Japanese
• Published: Japan 01-02-1998
• Subjects: Adult; Bone Marrow Transplantation - adverse effects; Busulfan - adverse effects; Chronic Disease; Cyclophosphamide - adverse effects; Cyclosporine - adverse effects; Graft vs Host Disease - complications; Hepatic Veno-Occlusive Disease - etiology; Hepatic Veno-Occlusive Disease - pathology; Humans; Immunosuppressive Agents - adverse effects; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - therapy; Male; Methotrexate - adverse effects; Recurrence; Respiratory Distress Syndrome, Adult - etiology; Tacrolimus - adverse effects
• ISSN: 0485-1439
• DOI: 10.11406/rinketsu.39.139

A 40-year-old man with chronic myelogenous leukemia in chronic phase received an allogeneic marrow graft from his HLA identical brother. He was conditioned with busulfan (16 mg/kg) and cyclophosphamide (120 mg/kg). Graft-versus-host disease (GVHD) prophylaxis was attempted with cyclosporine A (CYA) and methotrexate. On day 30, weight gain, ascites and hepatomegaly developed in addition to an elevation of total bilirubin (TB). He was diagnosed as having veno-occlusive disease (VOD) and treated conservatively. The TB level increased up to 20.1 mg/dl on day 66, then reduced to 2.1 mg/dl on day 129. By that time ascites and hepatomegaly also had completely resolved. However, on day 134. The TB level started to increase again, when the lesions of chronic GVHD were observed in the eye, the mouth, and the skin. CYA was started on day 142, and FK506 was substituted for CYA on day 161. Despite the improvement of oral and skin lesions, TB level continued to rise, and he died of respiratory failure due to ARDS on day 186. Autopsy revealed both acute and old hepatic VOD lesions, suggesting the occurrence of late-onset VOD which probably contributed to the liver dysfunction observed after clinical resolution of the first episode of VOD.