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台湾华裔人群中与I型人类嗜T细胞病毒相关的成人T细胞白血病/淋巴瘤

Human T-cell lymphotropic virus type I associated adult T-cell leukaemia/lymphoma in Taiwan Chinese.

作者信息

Shih L Y, Kuo T T, Dunn P, Liaw S J

机构信息

Department of Internal Medicine, Chang Gung Memorial Hospital, Chang Gung Medical College, Taipei, Taiwan, Republic of China.

出版信息

Br J Haematol. 1991 Oct;79(2):156-61. doi: 10.1111/j.1365-2141.1991.tb04516.x.

Abstract

Twenty-five Chinese patients with human T-cell lymphotropic virus type I (HTLV-I) associated adult T-cell leukaemia/lymphoma (ATLL) were identified in Taiwan. No patients had been outside Taiwan and none were descendants of Japanese heritage. Their ages ranged from 28 to 71 years. There were 17 men and eight women. Main clinical and laboratory features at presentation were lymphadenopathy (16), skin lesions (11), hepatosplenomegaly (11), pulmonary lesions (11), hypercalcaemia (10) and bone marrow infiltration (14). Peripheral blood was characterized by leucocytosis with presence of pleomorphic abnormal lymphocytes but rare anaemia or thrombocytopenia. The clinical subtypes were acute in 15, chronic in three, smouldering in one, and lymphoma type in six. The immunophenotypes of the ATLL cells were characterized by the expression of CD2+, CD4+, CD7-, CD8- and CD25+. The overall prognosis was poor with a median survival of 5 months. The acute form had a significantly shorter survival (2 months) than lymphoma type (13 months). Susceptibility to various infections was common. Pulmonary complications accounted for 73% of the causes of death. The clinicopathologic features of ATLL in Taiwan are indistinguishable from those in HTLV-I endemic areas. The present series adds to the knowledge of the worldwide pattern of the disease.

摘要

在台湾,共确诊了25例感染I型人类嗜T细胞病毒(HTLV-I)的成年T细胞白血病/淋巴瘤(ATLL)患者。所有患者均未曾离开过台湾,且均非日本裔后代。他们的年龄在28岁至71岁之间,其中男性17例,女性8例。初诊时的主要临床和实验室特征包括淋巴结病(16例)、皮肤病变(11例)、肝脾肿大(11例)、肺部病变(11例)、高钙血症(10例)和骨髓浸润(14例)。外周血的特征为白细胞增多,伴有多形性异常淋巴细胞,但贫血或血小板减少罕见。临床亚型为急性15例、慢性3例、冒烟型1例、淋巴瘤型6例。ATLL细胞的免疫表型特征为CD2+、CD4+、CD7-、CD8-和CD25+表达。总体预后较差,中位生存期为5个月。急性型的生存期(2个月)明显短于淋巴瘤型(13个月)。各种感染易感性常见。肺部并发症占死亡原因的73%。台湾ATLL的临床病理特征与HTLV-I流行地区的特征无明显差异。本系列病例增加了对该疾病全球模式的认识。

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