Published in:
Open Access
01-12-2017 | Case report
Portal hypertension as the initial manifestation of POEMS syndrome: a case report
Authors:
Lina Wu, Yue Li, Fang Yao, Chongmei Lu, Jian Li, Weixun Zhou, Jiaming Qian
Published in:
BMC Hematology
|
Issue 1/2017
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Abstract
Background
Portal hypertension has a broad differential diagnosis. POEMS syndrome is an uncommon cause of it. POEMS syndrome is a rare disease involving multiple organs. In differential diagnosis of portal hypertension, POEMS syndrome should be considered especially when other symptoms such as numbness, organomegaly, endocrine alteration and skin changes also present, as it is highlighted by our case.
Case presentation
We report a 46-year-old Chinese male, a teacher, presenting with portal hypertension. Electromyography revealed peripheral neuropathy. Immunofixation showed monoclonal immunoglobulin A lambda protein. The diagnosis of POEMS syndrome was established. After treatment of lenalidomide combined with dexamethasone over 2 years, the patient achieved a considerable improvement.
Conclusion
This case highlights the manifestation of portal hypertension in POEMS syndrome. Lenalidomide with or without dexamethasone is effective for portal hypertension due to POEMS syndrome, though esophageal and gastric varices seems not reversible so easily.