POEMS Syndrome Without “M”- Could It Be Possible? – A Case Report

Abstract

Introduction: POEMS syndrome is a rare disease characterized by polyneuropathy, organomegaly, endocrinopathy, “M-protein” disorder and skin changes. The purpose of this article is to report a case of a patient with progressive polyneuropathy without M protein and plasma cells disorder, one of the mandatory criteria diagnosis. Case: A 43 year-old man of Japanese origin with three months of weight loss, painful feet paresthesias and reduction of muscle strength in the lower limbs. At physical examination he presented distal motor deficit in legs, global absence of profound reflexes, feet amyotrophy, hepatosplenomegaly, gynecomastia, papilledema, axilar lymph node enlargement and a cyanosis of extremities. Myelogram and bone marrow biopsy discarded myeloma or plasma cell neoplasy. Protein electrophoresis and immunofixation electrophoresis not detected “M-spike”. After a clinical worsening, new diagnosis investigation was made and a vascular endothelial growth factor dosage was available (2812 pg/ml –normal range: 31-86pg/ml). Discussion: Polyneuropathy and plasma cells disorder are both mandatory diagnosis criteria for POEMS syndrome. However, we present a case without plasma cells disorder and “M-protein spike”, with the diagnosis confirmed with the combination of characteristic clinical manifestations and elevated vascular endothelial growth factor. We highlight that in some situations the diagnose can be made with auxiliaries exams to avoid delay in starting treatment.