Lazaro Estibaliz, Caubet Olivier, Menard Fanny, Pellegrin Jean-Luc, Viallard Jean-François
Service de médecine interne et maladies infectieuses, Centre François Magendie, Hôpital du Haut-Lévêque, Pessac.
Presse Med. 2007 Nov;36(11 Pt 2):1694-700. doi: 10.1016/j.lpm.2007.06.002. Epub 2007 Jun 26.
Large granular lymphocyte (LGL) leukemia is a clonal proliferation of cytotoxic cells, either CD3(+) (T-cell) or CD3(-) (natural killer, or NK). Both subtypes can manifest as indolent or aggressive disorders. T-LGL leukemia is associated with cytopenias and autoimmune diseases and most often has an indolent course and good prognosis. Rheumatoid arthritis and Felty syndrome are frequent. NK-LGL leukemias can be more aggressive. LGL expansion is currently hypothesized to be a virus (Ebstein Barr or human T-cell leukemia viruses) antigen-driven T-cell response that involves disruption of apoptosis. The diagnosis of T-LGL is suggested by flow cytometry and confirmed by T-cell receptor gene rearrangement studies. Clonality is difficult to determine in NK-LGL but use of monoclonal antibodies specific for killer cell immunoglobulin-like receptor (KIR) has improved this process. Treatment is required when T-LGL leukemia is associated with recurrent infections secondary to chronic neutropenia. Long-lasting remission can be obtained with immunosuppressive treatments such as methotrexate, cyclophosphamide, and cyclosporine A. NK-LGL leukemias may be more aggressive and refractory to conventional therapy.
大颗粒淋巴细胞（LGL）白血病是一种细胞毒性细胞的克隆性增殖，这些细胞可以是CD3(+)（T细胞）或CD3(-)（自然杀伤细胞，即NK细胞）。两种亚型都可表现为惰性或侵袭性疾病。T-LGL白血病与血细胞减少症和自身免疫性疾病相关，通常病程惰性，预后良好。类风湿性关节炎和费尔蒂综合征较为常见。NK-LGL白血病可能更具侵袭性。目前推测LGL扩增是一种病毒（爱泼斯坦-巴尔病毒或人类T细胞白血病病毒）抗原驱动的T细胞反应，涉及细胞凋亡的破坏。T-LGL的诊断通过流式细胞术提示，并通过T细胞受体基因重排研究得以证实。NK-LGL的克隆性难以确定，但使用针对杀伤细胞免疫球蛋白样受体（KIR）的单克隆抗体改善了这一过程。当T-LGL白血病与慢性中性粒细胞减少继发的反复感染相关时，需要进行治疗。使用甲氨蝶呤、环磷酰胺和环孢素A等免疫抑制治疗可获得持久缓解。NK-LGL白血病可能更具侵袭性，对传统治疗难治。
