POEMS Syndrome: an Atypical Presentation with Chronic Diarrhoea and Asthenia
Joana Alves Vaz1, Lilia Frada2, Maria Manuela Soares1
1Department of Internal Medicine, Egas Moniz Hospital, Lisbon, Portugal.
Abstract:
POEMS syndrome is a rare paraneoplastic condition associated with polyneuropathy, organomegaly, monoclonal gammopathy, endocrine and skin changes. We report a case of a man with Castleman disease and monoclonal gammopathy, with a history of chronic diarrhoea and asthenia. Gastrointestinal involvement in POEMS syndrome is not frequently referred to in the literature and its physiopathology is not fully understood. Diagnostic criteria were met during hospitalization but considering the patient's overall health condition, therapeutic options were limited. Current treatment for POEMS syndrome depends on the management of the underlying plasma cell disorder. This report outlines the importance of a thorough review of systems and a physical examination to allow an attempted diagnosis and appropriate treatment.
Learning Points:
POEMS syndrome should be suspected in the presence of peripheral polyneuropathy associated with monoclonal gammopathy; diagnostic workup is challenging and delay in treatment is very common.Gastrointestinal involvement is not frequent and initial presentation with chronic diarrhoea is unusual and rarely mentioned in the literature.There is no consensus on the therapeutic approach, which can include autologous transplantation and chemotherapy. Frequently, these patients present fatigue, weight loss and sarcopenia with reduction of performance status, limiting subsequent therapeutic options.
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