POEMS Syndrome: A Patient with an IgA lambda Plasma Cell Disease. Expression Levels of VEGF and bFGF Correlates with Clinical Course.

Purpose: POEMS syndrome is a rare disease characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. We present a severely ill woman with a four year history of polyneuropathy showing all signs of a POEMS syndrome. Response to chemotherapy including high-dose melphalan treatment and autologous bone marrow transplantation was monitored and vascular endothelial growth factor (VEGF) as well as basic fibroblast growth factor (bFGF) levels were measured.

Methods: Blood investigation was done for serum electrophoresis analysis and analysis of VEGF, bFGF and IL-6 by ELISA. Bone marrow biopsy specimen was investigated immunohistochemically for kappa, lambda, alpha, gamma, CD20, CD56, Cyclin D1, VEGF.

Results: Immunohistochemical investigation of the bone marrow biopsy showed a infiltration of lambda and alpha positive plasma cells (10%). Only few plasma cells expressed kappa. The tumor cell were negative for CD20, CD56 and Cyclin D1, but positive for VEGF in line with the high VEGF levels in the blood. Blood investigation revealed a discrete monoclonal gammopathy of IgA lambda type. Initially, high levels of VEGF (1468.7 pg/ml) and bFGF (112.9 pg/ml) were detected. However, treatment with high-dose melphalan and tandem autologous bone marrow transplantation proved extremely helpful in symptom control. Already after the first transplant the patient started to walk again and lost pulmonary hypertension. In parallel VEGF and bFGF levels decreased and the performance status of the patient improved dramatically.

Conclusion: VEGF and bFGF measurement is a useful tool for monitoring disease activity in POEMS syndrome. Bone marrow transplantation is an important therapy also in severely ill patients.