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.

Keywords: castleman disease; castleman variant of poems syndrome; plasma cell dyscrasia; poems syndrome; systemic autoimmune disease.

Figure 1. Bilateral claw hand with wasting of intrinsic muscles.

Figure 2. Bone marrow biopsy showing marked megakaryocytosis.

Figure 3. Bone marrow biopsy showing increased plasma cells (N <10%).

N: reference range

Figure 4. Lymph node biopsy showing marked vascular proliferation (Castleman disease).