纯红细胞再生障碍性贫血与T细胞大颗粒淋巴细胞白血病

纯红细胞再生障碍性贫血(PRCA)是骨髓红系细胞造血衰竭所导致的一类贫血,T淋巴细胞介导的自身免疫破坏机制被认为是PRCA的主要机制之一。T细胞型大颗粒淋巴细胞白血病(T-LGLL)常合并PRCA,在国内,已超过胸腺瘤成为PRCA最常见的合并症,白血病性细胞毒T淋巴细胞的增殖是导致骨髓红系造血衰竭的原因。本文对PRCA的发病机制、与T-LGLL的相关性、T-LGLL的发病和导致PRCA的机制以及T-LGLL的诊断、治疗、预后等方面的最新进展予以总结,以引起大家对二者相关性的认识和重视。

Pure red cell aplasia and T-cell large granular lymphocytic leukemia

Pure red cell aplasia （PRCA） is the diagnosis applied to isolated anemia secondary to failure of erythropoiesis. T-cell-mediated autoimmune destruction is considered to be one of the main mechanisms of PRCA. T-cell large granular lymphocytic leukemia （T-LGLL） is often associated with PRCA. In China, T-LGLL has become the most common complication of PRCA instead of thymoma. Proliferation of leukemic cytotoxic T lymphocytes is the cause of failure of erythropoiesis. In this article, the pathogenesis of PRCA, the pathogenesis and mechanism of T-LGLL with PRCA, and the latest developments lead to the diagnosis and treatment of the T-LGLL were summed to arouse attention to correlation between T-LGLL and PRCA.