Plumelle Y, Pascaline N, Nguyen D, Panelatti G, Jouannelle A, Jouault H, Imbert M
Hematology Laboratory, Regional Medical Center, Fort de France, Martinique.
Hematol Pathol. 1993;7(4):251-62.
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.
1983年至1991年间，在马提尼克岛（法属西印度群岛）确诊了26例成人T细胞白血病/淋巴瘤（ATLL）。其中男性14例，女性12例，均为混血后裔，出生于马提尼克岛。他们的年龄在23岁至95岁之间。初次就诊时的主要临床和实验室特征包括外周淋巴结病（22例）、肝肿大（11例）、脾肿大（10例）、皮肤病变（12例）、高钙血症（16例）、粪类圆线虫难治性感染（12例）以及既往存在的自身免疫性疾病（4例）。所有患者均有绝对淋巴细胞增多症及循环中多形性异常淋巴细胞。预后较差，大多数患者（20例）存活时间不足6个月。尽管本系列中ATLL的总体临床病理特征与既往报道相似，但我们观察到另外三个有趣的点：与粪类圆线虫感染高度相关、患者亲属中热带痉挛性轻瘫/HTLV-1相关脊髓病（TSP/HAM）的发病率增加（5例）以及既往存在胶原血管疾病。
