PLASMA CELL DYSCRASIA WITH POLYNEUROPATHY, ORGANOMEGALY, ENDOCRINOPATHY, M PROTEIN, and SKIN CHANGES: THE POEMS SYNDROME, ASSOCIATED WITH PRECEDING POLYCYTHEMIA VERA. A Case Report and Review of the Literature

A case of plasma cell dyscrasia with polyneuropathy and endocrine disorder is reported. Clinically, polycythemia vera, gynecomastia, pigmentation of the skin, hepatosplenomegaly, renal enlargement and severe polyneuropathy in the lower extremities were recognized. The peculiarity of this case was polycythemia vera that had been present for several years before manifestation of the clinical symptoms. Microscopically, retroperitoneal lymph nodes showed angio-follicular lymphoid hyperplasia and plasma cell infiltration in the inter-follicular region. By means of the avidin-biotin-peroxidase complex method, plasma cells were positive for A light chain, IgA and IgG. Severe segmental demyelination and slight axonal atrophy were found in a sural nerve biopsy.