Pulmonary veno-occlusive disease after hematopoietic stem cell transplantation
| Autor: | Kazuteri Ohashi, Hirotoshi Horio, Tsunekazu Hishima, Tatsuru Okamura, Makiko Yomota, Yusuke Ohkuma, Yukio Hosomi |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
medicine.medical_specialty
Lung business.industry medicine.medical_treatment Hematopoietic stem cell transplantation Lung biopsy medicine.disease Pulmonary hypertension Pulmonary function testing Surgery medicine.anatomical_structure Diffusing capacity Medicine Lung transplantation Pulmonary Veno-Occlusive Disease business |
| Zdroj: | 1.5 Diffuse Parenchymal Lung Disease. |
| Popis: | Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension with poor prognosis. PVOD may be idiopathic or associated with chronic diseases, but there are only 12 reported cases of PVOD following hematopoietic stem cell transplantation (HSCT). Two cases presenting in our hospital were pathologically diagnosed as PVOD following HSCT. Both cases presented with dyspnea on exertion and low SpO2. X-ray showed bilateral pleural effusion. On transthoracic echocardiography, ejection fraction was normal and there were no indications of pulmonary hypertension (PH) in either patient. Pulmonary function test revealed decreased diffusing capacity of the lung, and perfusion lung scintigraphy showed subsegmental defect. Surgical lung biopsy was performed, and fibrosis remodeling of the intima and occlusion of the lumen were observed, which are suggestive of a definitive PVOD diagnosis. Each case was treated with corticosteroid and their symptoms, SpO2, and pulmonary function improved. One patient died 281 days after diagnosis by bacterial pneumonia, and the other has been alive for 28 months and remains stable. PVOD prognosis is extremely poor, with most reported patients dying within 2 years of diagnosis. In previous reports of PVOD following HSCT, no patient has survived for more than 1 year, and all cases had PH. Four cases underwent surgical biopsy, while the others were diagnosed clinically or at autopsy. Nine patients were given corticosteroids as treatment, and four of these nine patients responded to the treatment. PVOD is rare, but early detection of this disease before the development of severe PH and early treatment or listing for lung transplantation may improve prognosis. |
| Databáze: | OpenAIRE |
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