Adult T-cell leukemia-lymphoma: a clinico-pathologic study of twenty-six patients from Martinique.

Hematologic pathology Pub Date : 1993-01-01
Y Plumelle, N Pascaline, D Nguyen, G Panelatti, A Jouannelle, H Jouault, M Imbert
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Abstract

Twenty-six cases of adult T-cell leukemia/lymphoma (ATLL) were identified between 1983 and 1991 in Martinique (French West Indies). There were 14 men and 12 women, all of mixed racial descent and born in Martinique. Their ages ranged from 23 to 95 years. The main clinical and laboratory features at initial presentation were peripheral lymphadenopathy (22 cases), hepatomegaly (11 cases), splenomegaly (10 cases), cutaneous lesions (12 cases), hypercalcemia (16 cases), refractory infection by Strongyloides stercoralis (12 cases), and pre-existing autoimmune disorders (4 cases). All patients had absolute lymphocytosis with circulating pleomorphic abnormal lymphocytes. The prognosis was poor, with most patients (20 cases) surviving for less than 6 months. Although the overall clinicopathologic features of ATLL in this series are similar to those described in previous reports, we observed three additional points of interest: a high association with Strongyloides infection, an increased incidence of tropical spastic paresis/HTLV-1 associated myelopathy (TSP/HAM) among the relatives of the patients (5 cases), and the presence of prior collagen vascular diseases.

成人t细胞白血病淋巴瘤:马提尼克岛26例患者的临床病理研究。
1983年至1991年间,在马提尼克岛(法属西印度群岛)发现了26例成人t细胞白血病/淋巴瘤(ATLL)。共有14名男性和12名女性,他们都是混血,出生在马提尼克岛。他们的年龄从23岁到95岁不等。首发时的主要临床和实验室特征为外周淋巴结病变(22例)、肝肿大(11例)、脾肿大(10例)、皮肤病变(12例)、高钙血症(16例)、难治性肠类圆杆菌感染(12例)和既往自身免疫性疾病(4例)。所有患者均有绝对淋巴细胞增多和循环多形性异常淋巴细胞。预后较差,多数患者(20例)生存时间不足6个月。尽管本系列中ATLL的总体临床病理特征与之前的报道相似,但我们观察到三个额外的兴趣点:与类圆线虫感染的高度关联,患者亲属中热带痉挛性麻痹/HTLV-1相关脊髓病(TSP/HAM)的发生率增加(5例),以及既往胶原血管疾病的存在。
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