Bortezomib plus dexamethasone as first-line therapy for patients with POEMS syndrome

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare plasma cell dyscrasia without standard front-line treatment. Merely, few studies have reported the responses and outcomes of bortezomib plus dexamethasone (BDex) in POEMS syndrome. In thi...

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Published inAnnals of hematology Vol. 100; no. 11; pp. 2755 - 2761
Main Authors Gao, Xue-min, Yu, Yan-ying, Zhao, Hao, Cai, Hao, Zhang, Lu, Cao, Xin-xin, Zhou, Dao-bin, Li, Jian
Format Journal Article
LanguageEnglish
Published Berlin/Heidelberg Springer Berlin Heidelberg 01.11.2021
Springer Nature B.V
Subjects
Drug dosages
Drug withdrawal
Electromyography
Hematology
Inhibitor drugs
Males
Medical schools
Medicine
Medicine & Public Health
Neurotoxicity
Oncology
Original Article
Patients
Peripheral neuropathy
Plasma
Pulmonary hypertension
Remission (Medicine)
Response rates
Steroids
Vascular endothelial growth factor
Time to next treatment
Front-line bortezomib
Overall survival
POEMS syndrome
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Summary:POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare plasma cell dyscrasia without standard front-line treatment. Merely, few studies have reported the responses and outcomes of bortezomib plus dexamethasone (BDex) in POEMS syndrome. In this study, a total of 69 patients (40 males) treated with front-line BDex were included. The median age at diagnosis was 50 years (range, 30–78 years). After a median of 9 cycles BDex (range 1–9), fifty-two (88.1%), thirty-two (46.4%), and forty-seven (71.2%) patients achieved the best neurologic response, hematological complete response, and serum vascular endothelial growth factor (VEGF) response, respectively. The extravascular overload, pulmonary hypertension, and renal impairment also substantially improved. No treatment-related death occurred. Two patients developed grade-1 bortezomib-induced peripheral neuropathy and were reversible after drug withdrawal. After a median follow-up of 22.5 months, the estimated 2-year overall survival and time to next treatment were 95.7% and 65.6%, respectively. In conclusion, the combination of bortezomib and dexamethasone is effective, with a high response rate and safety profile for patients with newly diagnosed POEMS syndrome.
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ISSN:0939-5555
1432-0584
DOI:10.1007/s00277-021-04609-6