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Short communication: Aggressive adult T cell leukemia/lymphoma: The tip of the iceberg of the hidden human T cell lymphotropic virus type 1 infection burden in nonendemic countries

机译:简短交流:侵略性成人T细胞白血病/淋巴瘤:非流行国家的1型人类T细胞淋巴病毒隐性感染负担的冰山一角

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Adult T cell leukemia/lymphoma has only rarely been reported in Europe. We aimed to determine the clinical characteristics and outcome of adult T cell leukemia/lymphoma patients in a nonendemic country. Cases of adult T cell leukemia/lymphoma managed at Hospital Universitari Vall d'Hebron, Barcelona, Spain were reviewed. Information on the foreign population living in Spain, according to country of origin, was obtained using official published data from the National Statistics Institute. Three patients were diagnosed with adult T cell leukemia/lymphoma between 2003 and 2010. Two cases were of the acute subtype and one case of the lymphoma subtype. Two patients were female and the mean age at presentation was 41.3 years. Patients originated from three different countries. The characteristics of the attended patients include widespread enlargement of the lymph nodes, a variety of multiple extranodal involvements, bone marrow infiltration, and a high incidence of infections including latent parasitic infections. Prototypic adult T cell leukemia/lymphoma presenting with high white cell counts, flower cells, and hypercalcemia was not observed. Regarding therapy, one patient received chemotherapy alone and two subjects combined first-line therapy including antiviral drugs. Of the three patients, two are dead (mean survival time 6 months) and one has been lost to follow-up. We estimate that at least 15,000 people living in Spain are infected with human T cell lymphotropic virus type 1 (HTLV-1). Adult T cell leukemia/lymphoma is a heterogeneous disease that often presents without distinguishing or prototypical features. A high index of clinical suspicion is essential for diagnosis. Several epidemiological differences have been observed in different countries. Today, HTLV-1 infection is highly underdiagnosed.
机译:在欧洲,鲜有成人T细胞白血病/淋巴瘤的报道。我们旨在确定一个非流行国家的成人T细胞白血病/淋巴瘤患者的临床特征和结局。回顾了西班牙巴塞罗那Universitari Vall d'Hebron医院管理的成人T细胞白血病/淋巴瘤病例。根据原籍国,使用西班牙国家统计局的官方公布数据来获得有关居住在西班牙的外国人口的信息。在2003年至2010年之间,三名被诊断为成人T细胞白血病/淋巴瘤。急性亚型2例,淋巴瘤亚型1例。两名患者为女性,出现时的平均年龄为41.3岁。患者来自三个不同的国家。照护患者的特征包括淋巴结广泛肿大,多种结外累及,骨髓浸润以及包括隐性寄生虫感染在内的高感染率。未观察到具有高白细胞计数,花细胞和高钙血症的原型成人T细胞白血病/淋巴瘤。关于治疗,一名患者仅接受化疗,两名受试者联合一线治疗,包括抗病毒药物。在三名患者中,有两人死亡(平均生存时间为6个月),其中一名已失去随访。我们估计,居住在西班牙的至少15,000人感染了1型人类T细胞淋巴病毒(HTLV-1)。成人T细胞白血病/淋巴瘤是一种异质性疾病,通常表现出没有明显的特征或典型特征。高临床怀疑指数对于诊断至关重要。在不同国家已经观察到几种流行病学差异。如今,HTLV-1感染被高度诊断不足。

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