Clinical characteristics and outcomes of 100 adult patients with pure red cell aplasia

被引：12

作者：

Wu, Xuemei ^{[1
]}

Cheng, Lang ^{[2
]}

Liu, Xiaoqing ^{[2
]}

Sun, Yu ^{[1
]}

Li, Bingzong ^{[1
]}

He, Guangsheng ^{[2
]}

Li, Jianyong ^{[2
]}

机构：

[1] Soochow Univ, Dept Hematol, Affiliated Hosp 2, Sanxiang Rd 1055, Suzhou 215004, Jiangsu, Peoples R China

[2] Nanjing Med Univ, Affiliated Hosp 1, Collaborat Innovat Ctr Canc Personalized Med, Jiangsu Prov Hosp,Key Lab Hematol,Dept Hematol, Guangzhou Rd 300, Nanjing 210029, Jiangsu, Peoples R China

来源：

ANNALS OF HEMATOLOGY | 2022年 / 101卷 / 07期

关键词：

Pure red cell aplasia; Secondary; Large granular lymphocytic leukemia; Cyclosporine A; GRANULAR LYMPHOCYTE LEUKEMIA; NATIONWIDE COHORT; IMMUNOSUPPRESSIVE THERAPY; ANEMIA; JAPAN; PATHOGENESIS; ASSOCIATION;

D O I：

10.1007/s00277-022-04847-2

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Adult pure red cell aplasia (PRCA) is a rare syndrome characterized by a severe normocytic anemia, reticulocytopenia, and absence of erythroblasts from bone marrow. The standard treatment has not yet been established for PRCA, although cyclosporine (CsA), corticosteroids (CS) showed a response in PRCA. We retrospectively analyzed the clinical data of 60 primary and 40 secondary adult patients with acquired PRCA. The proportion of secondary PRCA is relatively high and commonly associated with large granular lymphocyte leukemia (LGLL) (28 cases, 70.0%). The remission-induced regimens included CS, CsA, or other agents, and the response rate was 66.7%, 71.4%, and 50%, respectively (P = 0.336). When treating with CsA, the response rate of LGLL-associated PRCA was lower than primary PRCA (42.1% vs 85.7%, P = 0.001). Logistic regression analysis showed that ORR was inversely related to LGLL-associated PRCA. LGLL-associated PRCA had poor therapeutic efficacy to CsA.

引用

页码：1493 / 1498

页数：6

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