Qiu Zhi-Yuan, Qin Rong, Tian Guang-Yu, Wang Yan, Zhang Ye-Qing
Department of Oncology, The Affiliated People's Hospital of Jiangsu University, Zhenjiang 212002, Jiangsu, People's Republic of China.
Department of Vascular Surgery, The Second Affiliated Hospital of Soochow University, Suzhou 215004, Jiangsu, People's Republic of China.
Onco Targets Ther. 2019 Oct 4;12:8229-8240. doi: 10.2147/OTT.S222378. eCollection 2019.
Large granular lymphocytic leukemia (LGLL) is a chronic clonal lymphoproliferative disease of mature T or NK cells, and produces a variety of hematological abnormalities. Pure red cell aplasia (PRCA) is a rare haematological disease and is one of the most common complications of LGLL. LGLL-associated PRCA may represent a relatively indolent type and may be more common than reported, but its natural history and clinical course have not been well described. The ethnic origin of the patients is an important consideration in determining the relationship between PRCA and LGLL. Guidelines and progresses for management of LGLL-associated PRCA rely on accumulation of empirical experiences, integrative analyses of several cases and clinical trials. The purpose of this review is to evaluate occurrence, possible mechanisms, diagnosis, clinical features, treatments and outcomes of LGLL-associated PRCA.
大颗粒淋巴细胞白血病（LGLL）是一种成熟T或NK细胞的慢性克隆性淋巴细胞增殖性疾病，可产生多种血液学异常。纯红细胞再生障碍性贫血（PRCA）是一种罕见的血液学疾病，是LGLL最常见的并发症之一。LGLL相关的PRCA可能代表一种相对惰性的类型，可能比报道的更为常见，但其自然病史和临床过程尚未得到充分描述。患者的种族起源是确定PRCA与LGLL之间关系的重要考虑因素。LGLL相关PRCA的管理指南和进展依赖于经验积累、多例综合分析和临床试验。本综述的目的是评估LGLL相关PRCA的发生、可能机制、诊断、临床特征、治疗及预后。
