Chest

Chest

Volume 102, Issue 2, August 1992, Pages 642-643
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Selected Reports
Pulmonary Hypertension in Paroxysmal Nocturnal Hemoglobinuria

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Pulmonary arterial hypertension (PAH) and cor pulmonale were found in a patient with paroxysmal nocturnal hemoglobinuria (PNH). Autopsy revealed widespread thromboses in pulmonary microvasculature. Vascular thromboses attributed to hypercoagulability have been found in PNH in many organs, including the lungs. PAH has not been reported, however. This disease should then be considered a rare cause of PAH.

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CASE REPORT

A 53-year-old woman had the diagnosis of PNH made in 1955. Her disease started at the age of 18 years with hemolytic crises that became more frequent and intense in the following years, requiring periodic transfusions. In 1978, splenectomy was performed and since then, she has been treated intermittently with prednisone at doses ranging from 10 to 40 mg daily.

In 1981, she was first admitted to this hospital because of acute renal failure and she recovered after hemodialysis. In 1983, chest

DISCUSSION

Pulmonary hypertension developed in this patient with a 30-year history of PNH. During lifetime, common causes of pulmonary hypertension were excluded. At autopsy, thrombotic occlusion of the pulmonary microvaculature was demonstrated. Thrombotic pulmonary arteriopathy has been described as a histologic subset of primary pulmonary hypertension.4 Controversy exists as to whether these lesions arise from an embolic source or develop as a consequence of in situ thrombosis. There is little evidence

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Copyright © 1992 The American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.