1 research outputs found
Late-onset hepatic veno-occlusive disease post autologous peripheral stem cell transplantation successfully treated with oral defibrotide
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\u2032 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