Shichishima Tsutomu, Kawaguchi Michiko, Ono Nobutaka, Oshimi Kazuo, Nakamura Naoya, Maruyama Yukio
First Department of Internal Medicine, Fukushima Medical University, Fukushima, Japan.
Am J Hematol. 2004 Mar;75(3):168-72. doi: 10.1002/ajh.10480.
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.
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。