Successful Treatment with Bortezomib and Thalidomide for POEMS Syndrome Associated with Multicentric Mixed-type Castleman's Disease

• Published in: Japanese journal of clinical oncology Vol. 41; no. 10; pp. 1221 - 1224
• Main Authors: Wang, Xia, Ye, ShanHui, Xiong, ChunPing, Gao, JianQuan, Xiao, ChuYing, Xing, XiangBin
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.10.2011
• Subjects: Adult; Angiogenesis Inhibitors - administration & dosage; Angiogenesis Inhibitors - therapeutic use; Boronic Acids - administration & dosage; Boronic Acids - therapeutic use; Bortezomib; Castleman Disease - complications; Castleman Disease - immunology; Castleman Disease - pathology; Drug Therapy, Combination; Humans; Male; POEMS Syndrome - drug therapy; POEMS Syndrome - etiology; POEMS Syndrome - immunology; Pyrazines - administration & dosage; Pyrazines - therapeutic use; Thalidomide - administration & dosage; Thalidomide - therapeutic use; Vascular Endothelial Growth Factor A - antagonists & inhibitors; Vascular Endothelial Growth Factor A - blood; treatment; Castleman disease; VEGF; POEMS syndrome
• ISSN: 0368-2811; 1465-3621; 1465-3621
• DOI: 10.1093/jjco/hyr120

Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome is a rare multi-systematic disorder of uncertain etiology, if associated with multicentric Castleman's disease, it can lead to a more serious condition. We here presented a case of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome in a 37-year-old male patient who initially presented with progressive lower limb weakness accompanied by pain, low skin temperature, and hyperpigmentation. He was admitted with increasingly serious dyspnea and lower leg edema. Fluid of serous cavities in the patient were also indicated in ultrasonic inspection and X-ray. Furthermore, biopsy of a left axillary lymph node showed mixed hyaline-vascular and plasma cell type of multicentric Castleman's disease. Administration of bortezomib (Velcade) (1.3 mg/m2 on days 1, 4, 8 and 11 of a 21-day cycle) combined with thalidomide (100 mg/day and 21-day cycle) dramatically improved the condition of this disease. Of note, in our study, combination therapy of bortezomib and thalidomide successfully improved the condition of the patient with polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome associated with multicentric Castleman's disease, suggesting that the combination therapy may be an effective therapeutic strategy for the intractable polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome associated with multicentric Castleman's disease.