Analysis of a French cohort of patients with large granular lymphocyte leukemia: a report on 229 cases
Analysis of a French cohort of patients with large granular lymphocyte leukemia: a report on 229 cases
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DOI：
10.3324/haematol.2009.018481
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发表时间：
2010-09-01
期刊：
影响因子：
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通讯作者：
Lamy, Thierry
中科院分区：
文献类型：
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作者：
Bareau, Benoit;Rey, Jerome;Lamy, Thierry
背景大颗粒淋巴细胞白血病是一种罕见的淋巴组织增生性疾病，与自身免疫性疾病和造血功能受损有关。本研究描述了229例T细胞或NK细胞大颗粒淋巴细胞白血病的临床和生物学特征。设计和方法诊断基于大颗粒淋巴细胞扩增（> 0.5 × 10（9）/L）持续6个月以上。所有T细胞大颗粒淋巴细胞白血病均检测到单克隆T细胞受体γ基因重排。结果201例确诊为T细胞大颗粒淋巴细胞白血病，27例确诊为慢性NK细胞淋巴细胞增多症，1例确诊为NK细胞大颗粒淋巴细胞白血病。相关的自身免疫性疾病或其他肿瘤分别出现在74例和32例。100例患者（44%）需要治疗，主要是因为血小板相关感染（n=45）、症状性自身免疫疾病（n =24）、输血依赖性贫血（n=18）和其他原因（n=13）。患者接受类固醇（n= 33）、甲氨蝶呤（n=62）、环磷酰胺（n=32）或环孢霉素（n=24）作为一线、二线、三线或四线治疗。3个月时的总缓解率和各种治疗的完全缓解率如下：类固醇（12%和3%），甲氨蝶呤（55%和21%），环磷酰胺（66%和47%），环孢素（21%和4%），分别为。13例患者中有4例对脾切除术有反应。甲氨蝶呤治疗失败后，15例患者中有11例对环磷酰胺有反应。平均治疗次数为3.4（范围，1-7）。结论T细胞大颗粒淋巴细胞白血病和慢性NK细胞淋巴细胞增多症患者具有相似的临床、生物学特征和治疗反应。环磷酰胺的一线治疗应在前瞻性试验中进行测试。
Background Large granular lymphocyte leukemia is a rare lymphoproliferative disorder associated with autoimmune diseases and impaired hematopoiesis. This study describes the clinical and biological characteristics of 229 patients with T-cell or NK-cell large granular lymphocyte leukemia.Design and Methods The diagnosis was based on a large granular lymphocyte expansion (> 0.5x10(9)/L) lasting more than 6 months. Monoclonal T-cell receptor gamma gene rearrangement was detected in all the cases of T-cell large granular lymphocyte leukemia. Patients with chronic NK-cell lymphocytosis had an indolent disease, while those with multiorgan large granular lymphocyte infiltration and an aggressive clinical disease were considered to have NK-cell large granular lymphocyte leukemia.Results The diagnosis of T-cell large granular lymphocyte leukemia was confirmed in 201 cases, chronic NK-cell lymphocytosis in 27 cases and NK-cell large granular lymphocyte leukemia in one case. Associated auto-immune diseases or other neoplasms were present in 74 and 32 cases, respectively. One hundred patients (44%) required treatment, mainly for neutropenia-associated infections (n=45), symptomatic auto-immune diseases (n =24), transfusion-dependant anemia (n=18), and other causes (n=13). Patients were treated with steroids (n= 33), methotrexate (n=62), cytoxan (n=32), or cyclosporine (n=24) either as first-, second-, third- or fourth-line therapy. The overall response rate at 3 months and complete response rate for the various treatments were as follows: steroids (12% and 3%), methotrexate (55% and 21%), cytoxan (66% and 47%), cyclosporine (21% and 4%), respectively. Four out of 13 patients responded to splenectomy. Eleven out of 15 patients responded to cytoxan after methotrexate treatment had failed. The mean number of treatments was 3.4 (range, 1-7). There were 15 large granular lymphocyte leukemia-related deaths.Conclusions Patients with T-cell large granular lymphocyte leukemia and chronic NK-cell lymphocytosis have similar clinical and biological features and responses to treatment. First-line therapy with cytoxan should be tested in a prospective trial.