defibrotide and Pulmonary-Arterial-Hypertension

Pulmonary venoocclusive disease (PVOD) is a rare form of pulmonary vascular disease with pulmonary hypertension characterized by preferential involvement of the pulmonary venous system. Hepatic venoocclusive disease (HVOD), also known as sinusoidal obstruction syndrome, is a condition that occurs in 13% to 15% of patients after hematopoietic stem cell transplantation (HSCT). Although hepatic and pulmonary venoocclusive diseases may share some pathologic features as well as some etiologies such as HSCT, these two disorders have never been described together in a single adult patient. We report the case of a patient who received HSCT and developed HVOD and PVOD within 9 months. Despite their differences, PVOD and HVOD share common risk factors and associated conditions, suggesting that in the context of HSCT, the two diseases share common pathophysiological mechanisms. Optimal treatment for HSCT-related PVOD remains to be determined.

Topics: Ascites; Cardiac Catheterization; Endothelin Receptor Antagonists; Female; Fibrinolytic Agents; Hematopoietic Stem Cell Transplantation; Hepatic Veno-Occlusive Disease; Humans; Hyperbilirubinemia; Leukemia, Myeloid, Acute; Middle Aged; Phenylpropionates; Polydeoxyribonucleotides; Pulmonary Arterial Hypertension; Pulmonary Veno-Occlusive Disease; Pyridazines; Transplantation, Homologous; Treatment Outcome; Ultrasonography