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意大利的辅助性T细胞表型慢性淋巴细胞白血病和“成人T细胞白血病”。南欧地方性人嗜T淋巴细胞病毒I型相关T细胞白血病。

T-helper phenotype chronic lymphocytic leukaemia and "adult T-cell leukaemia" in Italy. Endemic HTLV-I-related T-cell leukaemias in southern Europe.

作者信息

Pandolfi F, Manzari V, De Rossi G, Semenzato G, Lauria F, Liso V, Ranucci A, Pizzolo G, Barillari G, Aiuti F

出版信息

Lancet. 1985 Sep 21;2(8456):633-6. doi: 10.1016/s0140-6736(85)90004-2.

Abstract

Sixteen Italian patients with chronic T-cell lymphocytic leukaemia (T-CLL) and leukaemic T-helper phenotype lymphocytes (Thp-CLL) were investigated for serum antibodies against human T-cell leukaemia virus I (HTLV-I) or its integrated DNA sequences. Common features of this series of patients were an aggressive clinical course with poor response to treatment, high white blood-cell count, bone-marrow infiltration, splenomegaly, and chromosome abnormalities. Three patients had skin infiltration and one had hypercalcaemia. Immunological analysis showed a Thp (OKT4+) in all cases, and a heterogeneity, within the OKT4 population, of phenotypes and functional activities. Three patients had either HTLV-I integrated DNA sequences or anti-HTLV-I serum antibodies, or both. These patients had not received any blood transfusions, denied intimate contact with foreigners, and had always lived in small towns of central or southern Italy. Clinical and immunological findings in this series of patients suggest that both HTLV-I related and unrelated cases of Thp-CLL should be regarded as one disease arising from the same subpopulation of mature T-lymphocytes.

摘要

对16例患有慢性T细胞淋巴细胞白血病(T-CLL)和白血病性T辅助表型淋巴细胞(Thp-CLL)的意大利患者进行了研究,检测其血清中针对人类T细胞白血病病毒I(HTLV-I)或其整合DNA序列的抗体。该系列患者的共同特征为临床病程侵袭性、对治疗反应不佳、白细胞计数高、骨髓浸润、脾肿大和染色体异常。3例患者有皮肤浸润,1例有高钙血症。免疫分析显示所有病例均为Thp(OKT4+),且在OKT4群体中,表型和功能活性存在异质性。3例患者要么有HTLV-I整合DNA序列,要么有抗HTLV-I血清抗体,或者两者都有。这些患者未曾接受过任何输血,否认与外国人有密切接触,且一直居住在意大利中部或南部的小镇。该系列患者的临床和免疫学发现表明,HTLV-I相关和不相关的Thp-CLL病例均应被视为源自同一成熟T淋巴细胞亚群的一种疾病。

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