POEMS Syndrome: A Case Report and Review of the Literature

Abstract

Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes (POEMS) syndrome is a rare multisystem disorder that occurs due to an underlying plasma cell dyscrasia. A diagnosis is made with the presence of two mandatory criteria and at least one major and one minor criterion. We present a case of a 28-year-old patient who presented with weakness of bilateral arms and legs, thinning of hands, and swelling of bilateral lower limbs and abdomen. The patient also reported weight loss and loss of appetite. Examination revealed areflexic quadriparesis with sensory loss, diffuse lymphadenopathy, pleural effusion, ascites, and pulmonary hypertension. Investigations showed elevated erythrocyte sedimentation rate (ESR). Nerve conduction studies revealed severe axonal polyneuropathy of all nerves. Lymph node biopsy showed Castleman disease. A diagnosis of POEMS syndrome was made and he was sent for a stem cell transplant, which is the definitive treatment in patients eligible for stem cell transplant.

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Plasma Cell Disorder, and Skin Changes) Syndrome as a Sequela of Castleman Disease: A Case Report

“…The diagnosis involves fulfilling two mandatory criteria: polyneuropathy (demyelinating) and monoclonal plasma cell proliferative disorder; one out of the following three major criteria: elevated vascular endothelial growth factor (VEGF), sclerotic bone lesions, and Castleman disease; and one of the following six minor criteria: organomegaly, endocrinopathy, extravascular volume overload, skin changes, papilledema, and thrombocytosis/polycythemia [ 6 ]. The diagnostic criteria are elaborated in Table 1 [ 2 ].…”

Neuroanatomical Changes and Visual Cognition in a Teenager with Cerebral Infarction – A Familial Case Study of POEMS Syndrome

“…A complete understanding of the association between POEMS syndrome and ischemic stroke has yet to be achieved [6,7]. Even with the novel consensus diagnostic criteria, it is still a rare and challenging condition to diagnose.…”

“…The relationship between POEMS syndrome and ischemic stroke, despite much research, remains elusive, with many aspects still not fully understood. [6][7][8][9][10][11]. POEMS syndrome also includes additional signi cant manifestations such as Castleman's disease, sclerotic bone lesions, papilledema, thrombocytosis, polycythemia, pulmonary hypertension, clubbing, weight loss, thrombotic diatheses, vitamin B12 de ciency, and extravascular volume overload.…”