大颗粒淋巴细胞白血病

Large Granular Lymphocyte Leukenia——Review

大颗粒淋巴细胞(LGL)白血病是一种少见的克隆性淋巴组织增殖性疾病。WHO分类将其区分为三种不同类型:T细胞大颗粒淋巴细胞白血病(T-LGL),NK-细胞的慢性淋巴增殖性疾病(CLPD-NK)和侵袭性NK细胞白血病。虽细胞起源不同,但T-LGL与CLPD-NK在临床表现和治疗方面很相似,多数患者无症状不需要治疗,当出现明显的血细胞减少时,首先考虑免疫抑制治疗。相反,侵袭性NK细胞白血病和罕见的CD56+侵袭性T-LGL白血病呈暴发性病程,发病年龄更早,需要较强的联合化疗序贯异基因造血干细胞移植。该类疾病相对稀少,故指导治疗的临床试验亦少。本文对该病的发病机制、诊断、治疗和预后进行了综述。

Large granular lymphocytic （LGL） leukaemia is an uncommon clonal lymphoproliferative disorder. The WHO classification recognizes three distinct disorders of LGLs： T-cell large granular lymphocytic leukaemia （T-LGL）, chronic lymphoproliferative disorders of NK-cells （CLPD-NK） and aggressive NK-cell leukaemia. Despite the different origin of cells, there is considerable overlap between T-LGL and CLPD-NK in terms of clinical presentation and treatment. Majority of these patients are asymptomatic and may not need treatment. When significant cytopenias occur, the application of irnmunosuppressive therapy often should be considered. In contrast, aggressive NK cell leukemia and the rare CD56 ＋ aggressive T-LGL leukemia have a fulminant clinical course and an earlier age of onset, therefore, more intensive combination chemotherapy is required, followed by allogeneic hematopoietic stem cell transplantation. However, these diseases are relatively rare, there are few clinical trials to guide management. In this review, the pathogenesis, diagnosis, treatment and prognosis of this leukemia are summarized and discussed.