Portal hypertension as the initial manifestation of POEMS syndrome: a case report

BMC Hematology
Lina WuJiaming Qian

Abstract

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. 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. 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.

References

Jul 31, 2012·Neurology·Kazuaki KanaiSatoshi Kuwabara
Jul 3, 2013·Annals of Hematology·Rong Tai CuiChuan Qiang Pu
Feb 18, 2014·American Journal of Hematology·Angela Dispenzieri
Mar 2, 2017·Revista Española De Enfermedades Digestivas : Organo Oficial De La Sociedad Española De Patología Digestiva·Sara CamposMaria Augusta Cipriano

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Methods Mentioned

BETA
electrophoresis
biopsy

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