Pulmonary veno-occlusive disease, a rare but fatal complication among patients with hematologic malignancy
Keywords:pulmonary veno-occlusive disease, hematopoietic stem cell transplantation, alkylating agent
Pulmonary veno-occlusive disease (PVOD), classified as group 1 pulmonary hypertension, is a rare but fatal form of pulmonary artery hypertension (PAH) manifesting subacute to chronic progressive dyspnea due to the increase in pulmonary arterial pressure and right-side heart failure. Exposure to chemotherapy and poststem cell transplantation constitute risk factors of PVOD. We herein demonstrate a case of PVOD in a middle-aged female with the underlying disease of T-cell/histiocyte-rich large B cell lymphoma treated using an alkylating agent and hematopoietic stem cell transplantation and presenting biventricular heart failure. After being treated using methylprednisolone, diuresis and pulmonary vasodilators, her clinical symptoms transiently improved. Unfortunately, the patient passed away one year after the diagnosis due to the progression of right-sided heart failure. Necropsy of the lung was performed revealing the occlusion of pulmonary venules.
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