九例成人T淋巴细胞白血病的临床与实验研究

Clinical and experimental study of 9 cases of adult T cell leukemia

目的探讨成人Ｔ淋巴细胞白血病（ＡＴＬ）的临床、细胞学、免疫学、细胞遗传学、血清学及分子生物学等特征。方法采用间接免疫荧光法及酶联免疫吸附法检测血清ＨＴＬＶⅠ抗体，建立ＰＣＲ和液相杂交法检测前病毒基因序列，结合ＭＩＣ特征确诊ＡＴＬ。结果和结论确诊９例ＡＴＬ，其临床特征主要为淋巴结肿大，皮损及溶骨改变少见，外周血及骨髓出现典型的花瓣状核淋巴细胞，胞体大，核畸形，免疫表型为ＣＤ－１、ＣＤ＋２、ＣＤ３＋、ＣＤ４＋、ＣＤ－８，还表达ＣＤ２５等激活标记，染色体核型及ＨＬＡ分型未发现有规律性的变化。８例ＡＴＬ中有７例血清ＨＴＬＶⅠ抗体阳性，应用ＰＣＲ及液相杂交法可检出整合到宿主细胞ＤＮＡ中的ＨＴＬＶⅠ病毒序列，表明在我国ＨＴＬＶⅠ也是ＡＴＬ的病因。９例中１例为淋巴瘤型，１例为慢性型，７例为急性型ＡＴＬ。

Objective In order to clarify the clinical and laboratory features of adult T cell leukemia (ATL): morphology, immunology, cytogenetics, serology and molecular biology. Methods Indirect immunofluorescence assay and ELISA were used to detect serum HTLV Ⅰ antibody. The HTLV Ⅰ provirus sequence were amplified by PCR and confirmed by liquid hybridization. Results and Conclusion Nine cases of ATL were diagnosed. The major clinical manifestation was lymph node enlargement found in all patients. Skin involvement and osteolysis were not frequent. The characteristic finding was leukemic cells with highly indented or lobulated flower like nuclei in peripheral blood and bone marrow. ATL cells were CD 2,CD 3,CD 4,CD 25 positive and CD 8 negative. No specific chromosome abnormality or HLA type was found. Seven of 8 patients examined had HTLV Ⅰ antibody. The HTLV Ⅰ provirus genome sequence integrated into host cell DNA was amplified by PCR and confirmed by liquid hybridization. All of these results showed that HTLV Ⅰ was also the etiological agent of ATL in China. One of the 9 cases of ATL was classified as lymphoma type, one as chronic type, and the rest as acute type.