Two cases of successful defibrotide treatment of sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell transplantation

Abstract

 Sinusoidal obstruction syndrome (SOS) is a fatal complication of hematopoietic stem cell transplantation. We report two cases of SOS where early defibrotide (DF) initiation led to recovery. The first case was that of a 49-year-old woman with Philadelphia chromosome-positive acute lymphoblastic leukemia. In the first complete remission, she underwent human leukocyte antigen (HLA) 2 antigen-mismatched cord blood transplantation, using a conditioning regimen with busulfan and cyclophosphamide. Organ damage and weight gain were observed on day 27 after transplantation, and she was diagnosed with SOS. DF was initiated on day 28 and her symptoms and collected data improved by day 54. The second case was that of a 67-year-old man with adult T-cell leukemia/lymphoma. In the first complete remission, he underwent HLA-matched unrelated peripheral blood hematopoietic stem cell transplantation, using a conditioning regimen with fludarabine and melphalan. Red blood cell fragmentation and transfusion-refractory thrombocytopenia were observed on day 8, followed by ascites and liver dysfunction. He was diagnosed with SOS and DF was administered on day 24. His conditions improved by day 36. Further studies are required to determine the appropriate timing of DF initiation.