Ixazomib-Based Regimen Followed by Autologous Hematopoietic Stem Cell Transplantation in the Treatment of POEMS Syndrome.
10.19746/j.cnki.issn.1009-2137.2022.03.044
- Author:
Ping CHENG
1
;
Lan-Lan WANG
1
;
Jun GUAN
1
;
Ying ZHOU
1
;
Qiu-Xiang WANG
1
;
Hui CHENG
1
;
Liang ZOU
2
Author Information
1. Department of Hematology, Wuhan No.1 Hospital, Wuhan 430022, Hubei Province, China.
2. Department of Hematology, Wuhan No.1 Hospital, Wuhan 430022, Hubei Province, China,E-mail: zozozou@qq.com.
- Publication Type:Journal Article
- Keywords:
POEMS syndrome;
autologous hematopoietic stem cell transplantation;
ixazomib;
proteasome inhibitor
- MeSH:
Adult;
Boron Compounds;
Glycine/analogs & derivatives*;
Hematopoietic Stem Cell Transplantation;
Humans;
Male;
Middle Aged;
POEMS Syndrome/therapy*;
Retrospective Studies;
Transplantation, Autologous;
Vascular Endothelial Growth Factor A
- From:
Journal of Experimental Hematology
2022;30(3):937-942
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE:To investigate the safety and efficacy of a new proteasome inhibitor Ixazomib followed by autologous hematopoietic stem cell transplantation (AHSCT) in the treatment of POEMS syndrome.
METHODS:The clinical manifestations, diagnosis and treatment process and follow-up results of 4 patients with POEMS syndrome who were treated with Ixazomib-based regimen combined with AHSCT in Wuhan No.1 Hospital from February 2018 to July 2020 were analyzed retrospectively. All patients were male, aged from 37-54 years old, with varying degrees of peripheral neuropathy, organ enlargement (liver, spleen or lymph nodes), circulatory overload (peripheral edema and/or pleural effusion), osteosclerosis, endocrine diseases (thyroid, gonads, etc.), skin changes (pigmentation, hemangioma, white nails, etc.), M protein, papilledema and other clinical manifestations and characteristics at the time of initial treatment. Two patients were pathologically diagnosed as hyaline vascular Castleman disease by lymph node biopsy. Three patients underwent lumbar puncture examinations and all showed elevated cerebrospinal fluid protein. All patients received at least 2 cycles of sequential AHSCT after induction chemotherapy based on ixazomib. The follow-up time was 10-28 months, and the median follow-up time was 16 months.
RESULTS:All cases survived. The complications were controllable during the treatment. Moreover, the clinical symptoms related to the disease were improved to a certain extent after the treatment. The levels of vascular endothelial growth factor (VEGF) showed a gradual decline.
CONCLUSION:Ixazomib combined with AHSCT is safe and effective in the treatment of POEMS syndrome.