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伴有自发缓解的γδ T细胞大颗粒淋巴细胞(LGL)白血病

Gammadelta T-cell large granular lymphocyte (LGL) leukemia with spontaneous remission.

作者信息

Shichishima Tsutomu, Kawaguchi Michiko, Ono Nobutaka, Oshimi Kazuo, Nakamura Naoya, Maruyama Yukio

机构信息

First Department of Internal Medicine, Fukushima Medical University, Fukushima, Japan.

出版信息

Am J Hematol. 2004 Mar;75(3):168-72. doi: 10.1002/ajh.10480.

Abstract

T-cell large granular lymphocyte (LGL) leukemia is a clonal disorder with an indolent clinical course. In July 1995, a 46-year-old Japanese man was admitted to our hospital because his anemia had progressed. He had a white blood cell count of 3.9 x 10(9)/L with 75% lymphocytes, which were intermediate to large and had almost round nuclei and azurophilic granules, and anemia with a red blood cell count (RBC) of 2.69 x 10(12)/L, hemoglobin (Hb) of 9.5 g/dL, and hematocrit (Hct) of 28.3%. Electron microscopic examination showed that most of the lymphocytes had a parallel tubular array and dense core granules in their cytoplasm. Flow cytometry and Southern blotting of the T-cell antigen receptor (TCR) genes using the peripheral blood species showed monoclonal proliferation of LGLs with a CD3+, TCRgammadelta+, CD4-, CD8-, CD16+, CD56-, CD57-, HLA-DR+ phenotype, and a TCR gamma gene rearrangement, respectively, suggesting that the patient was diagnosed as having gammadelta T-cell LGL leukemia. He had no symptoms, organomegaly, or skin lesions. About 1.5 years after diagnosis, the anemia gradually improved with disappearance and appearance of a rearranged band in the TCR-gamma gene and TCR-beta gene, respectively. About 7 years after diagnosis, the anemia improved completely with a RBC of 5.01 x 10(12)/L, Hb of 14.8 g/dL, and Hct of 44.3%, and he was in complete remission without TCR-beta and -gamma gene rearrangements. He had received no therapy. This is the first report of spontaneous remission of gammadelta T-cell LGL leukemia.

摘要

T细胞大颗粒淋巴细胞(LGL)白血病是一种临床病程进展缓慢的克隆性疾病。1995年7月,一名46岁的日本男性因贫血病情进展而入住我院。他的白细胞计数为3.9×10⁹/L,其中75%为淋巴细胞,这些淋巴细胞中等大小至大,细胞核几乎呈圆形,有嗜天青颗粒,同时伴有贫血,红细胞计数(RBC)为2.69×10¹²/L,血红蛋白(Hb)为9.5 g/dL,血细胞比容(Hct)为28.3%。电子显微镜检查显示,大多数淋巴细胞的细胞质中有平行管状排列和致密核心颗粒。对外周血进行T细胞抗原受体(TCR)基因的流式细胞术检测和Southern印迹分析,结果显示LGL呈单克隆增殖,其表型为CD3⁺、TCRγδ⁺、CD4⁻、CD8⁻、CD16⁺、CD56⁻、CD57⁻、HLA-DR⁺,且TCRγ基因发生重排,提示该患者被诊断为γδT细胞LGL白血病。他没有症状、器官肿大或皮肤病变。诊断后约1.5年,贫血逐渐改善,TCR-γ基因和TCR-β基因分别出现重排条带的消失和再现。诊断后约7年,贫血完全改善,红细胞计数为5.01×10¹²/L,血红蛋白为14.8 g/dL,血细胞比容为44.3%,且他处于完全缓解状态,TCR-β和-γ基因无重排。他未接受任何治疗。这是γδT细胞LGL白血病自发缓解的首例报告。

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