Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature.

IF 2.2 Q3 HEMATOLOGY

Anemia Pub Date : 2020-03-15 eCollection Date: 2020-01-01 DOI:10.1155/2020/4812759

Pimjai Niparuck, Wasana Kanoksil, Pathawut Wacharapornin, Pichika Chantrathammachart, Sarinya Boongird

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引用次数: 5

Abstract

Background: Pure red cell aplasia (PRCA) is less common blood disorder; the causes and the treatments of PRCA are varied.

Methods: We conducted a retrospective study during January 2010-December 2017, to explore the etiologies and to evaluate the response and treatment burden in adult patients with PRCA.

Results: Of 32 PRCA patients, median age was 57 years (18-90 years). Median hemoglobin level and reticulocyte count at the time of diagnosis were 5.6 g/dL (3.3-7.3 g/dL) and 0.3% (0.1-0.7%), respectively. Median time to hematologic recovery was 12 weeks (3-72 weeks), and median number of red blood cell transfusion (RBC) was 20 units (4-100 units). Causes of PRCA were erythropoiesis-stimulating agent (ESA) (47%), parvovirus B19 infection (19%), thymoma (13%), zidovudine (6%), primary autoimmune PRCA (6%), Kaposi's sarcoma (3%), systemic lupus erythematosus (3%), and ABO-mismatched stem cell transplantation (3%). Only 9 out of 24 treated patients achieved hematologic response within 8 weeks of treatment. Intravenous immunoglobulin therapy provided 100% response rate in patients with parvovirus B19-associated PRCA and primary autoimmune PRCA. Low response rate was found in patients receiving immunosuppressants and chemotherapy for the treatment of ESA and thymoma-associated PRCA, respectively.

Conclusions: Treatment outcome of PRCA depended upon the causes and the types of treatment, and the burden of RBC transfusion was very high in patients with ESA and thymoma-associated PRCA.

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成人纯红细胞发育不全患者的病因和治疗负担:单中心经验和文献综述。

背景:纯红细胞发育不全(PRCA)是一种少见的血液疾病;PRCA的病因和治疗方法多种多样。方法:我们于2010年1月至2017年12月进行了一项回顾性研究，探讨成人PRCA患者的病因，并评估其疗效和治疗负担。结果:32例PRCA患者中位年龄为57岁(18-90岁)。诊断时血红蛋白水平和网织红细胞计数中位数分别为5.6 g/dL (3.3-7.3 g/dL)和0.3%(0.1-0.7%)。血液学恢复的中位数时间为12周(3-72周)，红细胞输血(RBC)的中位数为20个单位(4-100个单位)。PRCA的病因为促红细胞生成剂(ESA)(47%)、细小病毒B19感染(19%)、胸腺瘤(13%)、齐多夫定(6%)、原发性自身免疫性PRCA(6%)、卡波西氏肉瘤(3%)、系统性红斑狼疮(3%)和abo -错配干细胞移植(3%)。24名接受治疗的患者中只有9名在治疗8周内达到血液学反应。静脉注射免疫球蛋白治疗对细小病毒b19相关PRCA和原发性自身免疫性PRCA患者的有效率为100%。在接受免疫抑制剂和化疗治疗ESA和胸腺瘤相关PRCA的患者中，分别发现低有效率。结论:PRCA的治疗结果与病因和治疗方式有关，ESA合并胸腺瘤相关PRCA患者的红细胞输血负担很高。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Anemia HEMATOLOGY-

CiteScore

4.80

自引率

3.40%

发文量

审稿时长

18 weeks

期刊介绍： Anemia is a peer-reviewed, Open Access journal that publishes original research articles, review articles, and clinical studies on all types of anemia. Articles focusing on patient care, health systems, epidemiology, and animal models will be considered, among other relevant topics. Affecting roughly one third of the world’s population, anemia is a major public health concern. The journal aims to facilitate the exchange of research addressing global health and mortality relating to anemia and associated diseases.