title: Late-onset hepatic veno-occlusive disease post autologous peripheral stem cell transplantation successfully treated with oral defibrotide.
abstract: Hepatic veno-occlusive disease (VOD) remains one of the commonest and most serious complications after myeloablative hematopoietic stem cell transplantation (HSCT). Clinical diagnosis of hepatic VOD is based on the finding of the triad of painful hepatomegaly, hyperbilirubinemia, and unexplained fluid retention occurring within 21 days of the transplant. However, the uncommon clinical entity of late-onset VOD can occur even beyond 20 days and should be considered in the differential diagnosis of any liver disease of more than 3 weeks' duration. While mild cases usually resolve spontaneously, severe VOD is associated with a grim prognosis. Defibrotide, a polydisperse mixture of single-stranded oligonucleotide with antithrombotic and fibrinolytic effects on microvascular endothelium, has emerged as an effective and safe therapy for patients with severe VOD. We describe a patient who presented 55 days post transplant with clinical features suggestive of VOD. Upon treatment with oral defibrotide, he showed complete resolution of the VOD.
authors.affiliation: BMT Unit, Department of Medical Oncology, Advanced Center for Treatment, Research and Education in Cancer (ACTREC), Tata Memorial Center, Kharghar, Navi Mumbai-410 208, Maharashtra, India.
authors.forename: Mithun S
authors.initials: MS
authors.lastname: Shah
authors.forename: Nandish Kumar S
authors.initials: NK
authors.lastname: Jeevangi
authors.forename: Amit
authors.initials: A
authors.lastname: Joshi
authors.forename: Navin
authors.initials: N
authors.lastname: Khattry
mesh_terms: D000293:Adolescent; D005343:Fibrinolytic Agents / Q000627:therapeutic use*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006504:Hepatic Veno-Occlusive Disease / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D008297:Male; D011089:Polydeoxyribonucleotides / Q000627:therapeutic use*; D012512:Sarcoma, Ewing / Q000601:surgery; D013995:Time
chemical_list: D005343:Fibrinolytic Agents; D011089:Polydeoxyribonucleotides; C036901:defibrotide