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Case Reports in Medicine
Volume 2012 (2012), Article ID 836893, 7 pages
http://dx.doi.org/10.1155/2012/836893
Case Report

Severe Pulmonary Hypertension Caused by Smoldering Plasma Cell Myeloma: An Autopsy Case of POEMS Syndrome

1Department of Pathology, Kyorin University School of Medicine, 6-20-2 Shinkawa, Tokyo, Mitaka 181-8611, Japan
2Department of Pathology, Nerima General Hospital, 1-24-1 Asahigaoka, Tokyo, Nerima 176-8530, Japan

Received 9 August 2012; Revised 7 November 2012; Accepted 8 November 2012

Academic Editor: Raed Dweik

Copyright © 2012 Katsuya Chinen and Yasunori Fujioka. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is a rare variant of plasma cell disorders with multiple systemic manifestations. Recently, pulmonary hypertension (PH) has become established as a complication, but pathological studies of this condition are scarce and the detailed pathogenesis remains to be elucidated. We present herein a case of a 49-year-old woman who was diagnosed as having idiopathic PH and was treated in accordance. However, she eventually died of respiratory failure and an autopsy revealed the presence of smoldering plasma cell myeloma and multiple organomegaly in addition to severe PH. The latter was attributed to stenosis and occlusion of the arterioles of the lungs due to marked plasma cell proliferation, quite different from the histology of idiopathic PH. From these findings, together with the clinical details, we concluded that the patient’s PH was a complication of the POEMS syndrome. This case showed a unique pulmonary vascular pathology featuring plasma cell proliferation and it provides clues towards understanding the pathogenesis with this background.