Guisnel Charles, Lesesve Jean-François, Gérard Delphine, Salignac Sylvain, Muller Marc, Feugier Pierre, Broséus Julien, Latger-Cannard Véronique
Service d'hématologie biologique, CHRU de Nancy, France, Service d'hématologie clinique et médecine interne, CHRU de Nancy, France.
Service d'hématologie biologique, CHRU de Nancy, France, Plateforme de cytométrie en flux, CHRU de Nancy, France, Unité Inserm U1256 NGERE, Faculté de médecine, Université de Lorraine, Vandoeuvre, France.
Ann Biol Clin (Paris). 2022 Feb 1;80(1):47-54. doi: 10.1684/abc.2022.1701.
Large granular lymphocytic leukemia (LGLL) is a rare clonal lymphoproliferative disorder from T or NK origin.
to report on the diagnostic and therapeutic management of LGLL investigated in the university hospital at Nancy, France.
retrospective (7 years) collection of clinical and biological data and patients' cohort analysis.
Eight out of fifteen patients presented with neutropenia, including five profound neutropenia (neutrophils < 500 × 10/L). Four patients had an infection. Two patients have rheumatoid arthritis and an associated Felty's syndrome, one a Sweet syndrome. Two also suffered from chronic Lymphocytic Leukemia, and one from a diffuse large B-cell lymphoma. Twelve patients had LGLL-T and 3 had a chronic LGLL-NK. Eleven out of twelve patients had a clonal LGLL-T when polymerase chain reaction assessed. No KIR clonality was sought among the 3 LGL-NK patients. Five patients out of fifteen received immunosuppressive treatment.
Although using simple and robust investigations, our series demonstrates a high heterogeneity in LGLL detection and assessment.
大颗粒淋巴细胞白血病(LGLL)是一种罕见的起源于T或NK细胞的克隆性淋巴细胞增殖性疾病。
报告在法国南锡大学医院研究的LGLL的诊断和治疗管理情况。
回顾性(7年)收集临床和生物学数据并进行患者队列分析。
15例患者中有8例出现中性粒细胞减少,其中5例为严重中性粒细胞减少(中性粒细胞<500×10⁹/L)。4例患者发生感染。2例患者患有类风湿性关节炎及相关的费尔蒂综合征,1例患有斯威特综合征。2例还患有慢性淋巴细胞白血病,1例患有弥漫性大B细胞淋巴瘤。12例患者为T细胞型LGLL,3例为慢性NK细胞型LGLL。在进行聚合酶链反应评估时,12例患者中有11例为克隆性T细胞型LGLL。3例NK细胞型LGL患者未进行KIR克隆性检测。15例患者中有5例接受了免疫抑制治疗。
尽管采用了简单可靠的检查方法,但我们的系列研究表明LGLL的检测和评估存在高度异质性。