POEMS syndrome and Waldenström's macroglobulinaemia

• Published in: Journal of clinical pathology Vol. 49; no. 2; pp. 181 - 182
• Main Authors: Pavord, S R, Murphy, P T, Mitchell, V E
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Association of Clinical Pathologists 01.02.1996; BMJ; BMJ Publishing Group LTD
• Subjects: Biological and medical sciences; Hematologic and hematopoietic diseases; Humans; Immunoglobulin kappa-Chains - blood; Immunoglobulin M - blood; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Male; Medical sciences; Middle Aged; Paraproteinemias - complications; POEMS Syndrome - etiology; Waldenstrom Macroglobulinemia - complications; Case study; Human; Immunopathology; Kappa light peptide chain; Immunoglobulinopathy; Lymphoproliferative syndrome; Malignant hemopathy; Waldenstrom macroglobulinemia; Complex syndrome; IgM
• ISSN: 0021-9746; 1472-4146
• DOI: 10.1136/jcp.49.2.181

A 58 year old man presented with a three year history of impotence, night sweats and ankle swelling. On examination, the patient fulfilled the diagnostic criteria for POEMS syndrome, but was unusual in that he also had underlying Waldenström's macroglobulinaemia with IgM kappa paraproteinaemia. The patient was treated with intermittent chlorambucil and made a good recovery. POEMS syndrome has been described in association with osteosclerotic myeloma and Castleman's disease. The paraprotein involved is usually IgG or IgA with lambda light chains. This case indicates that the presence of lambda light chains is not essential for the pathogenesis of POEMS syndrome. It also emphasises the diversity of plasma cell dyscrasias that can manifest as POEMS syndrome.