纯红细胞再生障碍性贫血的诊断与治疗进展  

Progress in Diagnosis and Treatment of Pure Red Cell Aplastic Anemia

作　　者：王兰兰 郝建萍[1] 

机构地区：[1]新疆医科大学第一附属医院血液科,新疆 乌鲁木齐

出　　处：《临床医学进展》2024年第1期2058-2063,共6页Advances in Clinical Medicine

摘　　要：纯红细胞发育不全(PRCA)是一种罕见的血液学综合征,其特征是孤立的正红细胞贫血伴严重网状红细胞减少症,并以骨髓中红细胞前体缺失或几乎缺失为特征。PRCA可能是一种原发性自身免疫性或克隆性髓系或淋巴系疾病,但也可能继发于其他自身免疫疾病、感染、肿瘤或药物。PRCA导致的血细胞异常仅限于红细胞谱系,通常不影响其他细胞系。从PRCA研究中获得的见解有助于阐明对红细胞生成调控的理解。这篇综述总结了PRCA的分类、诊断和治疗方法。Pure red cell aplasia (PRCA) is a rare hematologic syndrome, characterized by an isolated normo-cytic anemia with severe reticulocytopenia, and defined by absence or near absence of erythroid precursors in the bone marrow. PRCA may be a primary autoimmune or clonal myeloid or lymphoid disorder, but may also be secondary to other disorders of autoimmunity, to infections, to neoplasms, or to drugs. Insights from the study of PRCA have helped illuminate the understanding of the regu-lation of erythropoiesis. This review summarizes the classification, diagnosis, and treatment of PRCA.

关 键 词：纯红细胞再生障碍性贫血 克隆造血 环孢素 西罗莫司 

分 类 号：R55[医药卫生—血液循环系统疾病]