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On-line Access: 2021-08-20

Received: 2020-09-12

Revision Accepted: 2020-12-23

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Citations:  Bibtex RefMan EndNote GB/T7714

 ORCID:

He HUANG

https://orcid.org/0000-0002-2723-1621

Yanmin ZHAO

https://orcid.org/0000-0003-3857-5574

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Journal of Zhejiang University SCIENCE B 2021 Vol.22 No.8 P.695-700

http://doi.org/10.1631/jzus.B2000532


Successful treatment of refractory pure red cell aplasia with eltrombopag after ABO-incompatible allogeneic hematopoietic stem cell transplantation


Author(s):  Yang GAO, Fei GAO, Jimin SHI, Huarui FU, He HUANG, Yanmin ZHAO

Affiliation(s):  Bone Marrow Transplantation Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, China; more

Corresponding email(s):   yanminzhao@zju.edu.cn, huanghe@zju.edu.cn

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Yang GAO, Fei GAO, Jimin SHI, Huarui FU, He HUANG, Yanmin ZHAO. Successful treatment of refractory pure red cell aplasia with eltrombopag after ABO-incompatible allogeneic hematopoietic stem cell transplantation[J]. Journal of Zhejiang University Science B, 2021, 22(8): 695-700.

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author="Yang GAO, Fei GAO, Jimin SHI, Huarui FU, He HUANG, Yanmin ZHAO",
journal="Journal of Zhejiang University Science B",
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pages="695-700",
year="2021",
publisher="Zhejiang University Press & Springer",
doi="10.1631/jzus.B2000532"
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%0 Journal Article
%T Successful treatment of refractory pure red cell aplasia with eltrombopag after ABO-incompatible allogeneic hematopoietic stem cell transplantation
%A Yang GAO
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%A Huarui FU
%A He HUANG
%A Yanmin ZHAO
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%I Zhejiang University Press & Springer
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A1 - Yang GAO
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A1 - Huarui FU
A1 - He HUANG
A1 - Yanmin ZHAO
J0 - Journal of Zhejiang University Science B
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DOI - 10.1631/jzus.B2000532


Abstract: 
Pure red cell aplasia (PRCA) is a well-recognized complication of ABO major mismatched allogeneic hematopoietic stem cell transplantation (allo-HSCT), with a reported incidence of 10%–20% (Zhidong et al., 2012; Busca et al., 2018). It is clinically characterized by anemia, reticulocytopenia, and the absence of erythroblasts in a normal-appearing bone marrow biopsy (Shahan and Hildebrandt, 2015). The mechanism for PRCA has been presumed to be persistence of recipient isoagglutinins, produced by residual host B lymphocytes or plasma cells, which can interfere with the engraftment of donor erythroid cells (Zhidong et al., 2012). Several risk factors of PRCA at presentation are known, such as presence of anti-A isoagglutinins before transplantation, reduced intensity conditioning, absence of acute graft-versus-host disease (GVHD), sibling donors, and cyclosporin A (CsA) as GVHD prophylaxis (Hirokawa et al., 2013). PRCA is not considered to be a barrier to HSCT, as some patients can recover spontaneously or benefit from various approaches including high-dose steroids, erythropoietin (EPO), plasma exchange, immunoadsorption, donor lymphocyte infusion (DLI), treatment with rituximab, bortezomib, or daratumumab, and tapering or discontinuation of immunosuppression (Hirokawa et al., 2013; Bathini et al., 2019). However, there are still some patients who fail to respond even to aggressive treatment; they become red cell transfusion-dependent and iron-overloaded, and their life quality is impaired.

艾曲波帕成功治疗ABO血型不合的异基因造血干细胞移植后难治性纯红细胞再生障碍性贫血

摘要:纯红细胞再生障碍性贫血是ABO血型不合的异基因造血干细胞移植后常见的并发症。治疗该并发症有多种方法可供选择,包括大剂量激素、促红细胞生成素、血浆置换、免疫吸附、供者淋巴细胞输注、利妥昔单抗、硼替佐米、达雷木单抗以及减停免疫抑制剂等。在本文中,我们呈现了一个ABO血型不合的异基因造血干细胞移植后出现纯红细胞再生障碍性贫血的病例。重组人促红细胞生成素、糖皮质激素、供着淋巴细胞输注、血浆置换以及减停免疫抑制剂等方法均对其无明显疗效,最终在足剂量长疗程的艾曲波帕治疗下,患者得以康复。通过对艾曲波帕在治疗纯红细胞再生障碍性贫血中的机制的推测,我们发现艾曲波帕可能对单系造血有效,并且可被用于ABO不合的异基因造血干细胞移植后纯红细胞再生障碍性贫血的治疗。

关键词:纯红细胞再生障碍性贫血;异基因造血干细胞移植;艾曲波帕

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Reference

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