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侵袭性成人T细胞白血病/淋巴瘤:非流行国家中隐匿的人类嗜T细胞病毒1型感染负担的冰山一角。

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.

作者信息

Lopez-Lerma Ingrid, Caballero Estrella, Palacio Carlos, Garcia-Patos Vicente

机构信息

Department of Dermatology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

出版信息

AIDS Res Hum Retroviruses. 2013 Apr;29(4):704-8. doi: 10.1089/AID.2012.0185. Epub 2012 Dec 26.

Abstract

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.

摘要

成人T细胞白血病/淋巴瘤在欧洲仅有极少的病例报道。我们旨在确定一个非流行国家中成人T细胞白血病/淋巴瘤患者的临床特征及预后情况。对西班牙巴塞罗那市瓦尔德希伯伦大学医院收治的成人T细胞白血病/淋巴瘤病例进行了回顾。根据西班牙国家统计局官方发布的数据,获取了在西班牙居住的外国人口按原籍国划分的信息。2003年至2010年间,有3例患者被诊断为成人T细胞白血病/淋巴瘤。其中2例为急性亚型,1例为淋巴瘤亚型。2例患者为女性,就诊时的平均年龄为41.3岁。患者来自3个不同的国家。这些就诊患者的特征包括淋巴结广泛肿大、多种结外受累、骨髓浸润以及包括潜伏性寄生虫感染在内的感染发生率较高。未观察到典型的成人T细胞白血病/淋巴瘤表现为高白细胞计数、花细胞及高钙血症。在治疗方面,1例患者仅接受了化疗,2例患者接受了包括抗病毒药物在内的一线联合治疗。3例患者中,2例已死亡(平均生存时间6个月),1例失访。我们估计,至少有15000名居住在西班牙的人感染了1型人类T淋巴细胞病毒(HTLV-1)。成人T细胞白血病/淋巴瘤是一种异质性疾病,常常没有典型特征或区别性表现。高度的临床怀疑指数对诊断至关重要。不同国家已观察到一些流行病学差异。如今,HTLV-1感染的诊断严重不足。

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