Uchiyama T, Takatsuki K
Gan To Kagaku Ryoho. 1983 Feb;10(2 Pt 2):642-9.
Adult T cell leukemia (ATL) is a new disease entity with the following characteristic clinical and hematologic flutures: 1) Acute or subacute T cell leukemia in adulthood 2) Endemic in Kyushu and Southwest of Shikoku 3) Frequent skin infiltration 4) Common hepatosplenomegaly and lymphadenopathy (not so marked) 5) Mild or moderate bone marrow infiltration 6) Hypercalcemia 7) Typical leukemia cells with deeply-indented or lobulated nuclei and heterogeneous(pleomorphic) cells 8) No mediastinal mass 9) Ineffective ordinary treatment and short survival time Surface phenotypes of ATL cells were OKT 3(+) 4(+) 5(-) 8(-) Tac(+) and leukemia cells suppressed PWM-induced Ig synthesis in about half cases. Chromosome analysis showed the high incidence of abnormalities such as trisomy 7 and 14q +. ATL was compared with cutaneous T cell leukemia, T-cell chronic lymphocytic leukemia and lymphosarcoma cell leukemia in clinical features, hematologic and immunologic characteristics and the association with type C retrovirus.
成人T细胞白血病(ATL)是一种新的疾病实体,具有以下特征性的临床和血液学特点:1)成人急性或亚急性T细胞白血病;2)在九州和四国西南部地区呈地方性流行;3)常有皮肤浸润;4)常见肝脾肿大和淋巴结病(不太明显);5)骨髓呈轻度或中度浸润;6)高钙血症;7)典型的白血病细胞,核有深切迹或分叶,细胞呈异质性(多形性);8)无纵隔肿块;9)常规治疗无效,生存期短。ATL细胞的表面表型为OKT 3(+)、4(+)、5(-)、8(-)、Tac(+),约半数病例中白血病细胞可抑制PWM诱导的免疫球蛋白合成。染色体分析显示异常发生率较高,如7号染色体三体和14q +。对ATL与皮肤T细胞白血病、T细胞慢性淋巴细胞白血病及淋巴肉瘤细胞白血病在临床特征、血液学和免疫学特点以及与C型逆转录病毒的关联方面进行了比较。