High incidence of Epstein-Barr virus (EBV)-positive Hodgkin lymphoma and Hodgkin lymphoma-like B-cell lymphoproliferations with EBV latency profile 2 in children with interleukin-2-inducible T-cell kinase deficiency
High incidence of Epstein-Barr virus (EBV)-positive Hodgkin lymphoma and Hodgkin lymphoma-like B-cell lymphoproliferations with EBV latency profile 2 in children with interleukin-2-inducible T-cell kinase deficiency
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DOI：
10.1111/his.12677
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发表时间：
2015-11-01
期刊：
影响因子：
6.4
通讯作者：
Oschlies, Ilske
中科院分区：
文献类型：
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作者：
Bienemann, Kirsten;Borkhardt, Arndt;Oschlies, Ilske
目的：白细胞介素-2诱导的t细胞激酶（ITK）缺乏症是一种遗传性t细胞缺乏症，以eb病毒（EBV）相关淋巴细胞增生为特征。我们的目的是描述ITK缺乏引起的淋巴细胞增生过程的组织病理学特征，并将其与免疫功能低下患者的淋巴细胞增生进行比较。方法和结果根据世界卫生组织标准，对7例itk缺陷儿童的12例淋巴组织增生活检进行病理诊断，并通过EBV编码的小RNA、潜伏膜蛋白1、EBV核抗原2和ZEBRA染色确定EBV潜伏期类型和裂解活性。我们发现多形性和交界性多形性到单形性的b细胞增生，在大细胞中有不同的内容（5例），一个霍奇金样b细胞增生（1例），和典型的混合细胞霍奇金淋巴瘤（6例）。所有病例（12/12）均为ebv阳性。在典型的EBV阳性霍奇金淋巴瘤中，霍奇金淋巴瘤样和霍奇金淋巴瘤以及除一例外的所有多态b细胞淋巴增生均表现为EBV潜伏期2型。结论100%的EBV相关性、EBV阳性的经典霍奇金淋巴瘤和霍奇金样b细胞增殖的高比例以及EBV潜伏期2型甚至在多形病变中占主导地位是ITK缺乏患者淋巴细胞增殖的主要特征，提示这种t细胞免疫缺陷中淋巴瘤形成的独特病理机制。
AimsInterleukin-2-inducible T-cell kinase (ITK) deficiency is an inherited T-cell deficiency characterized by the development of Epstein-Barr virus (EBV)-associated lymphoproliferations. We aimed to describe the histopathological features of lymphoproliferative processes arising in ITK deficiency, and to compare them with lymphoproliferations in otherwise immunocompromised patients.Methods and resultsWe revised the histopathological diagnoses of 12 biopsies of lymphoproliferations from seven ITK-deficient children according to the World Health Organization criteria, and determined the EBV latency types and lytic activity by staining for EBV-encoded small RNA, latent membrane protein 1, EBV nuclear antigen 2, and ZEBRA. We found polymorphic and borderline polymorphic to monomorphic B-cell lymphoproliferations with variable contents in large cells (five cases), a Hodgkin-like B-cell proliferation (one case), and classic mixed-cellularity Hodgkin lymphoma (six cases). All cases (12/12) were EBV-positive. The Hodgkin lymphoma-like and Hodgkin lymphoma, and all but one polymorphic B-cell lymphoproliferation, showed EBV latency type 2, as observed in classic EBV-positive Hodgkin lymphoma.ConclusionsThe 100% EBV association, the high percentage of EBV-positive classic Hodgkin lymphoma and Hodgkin-like B-cell proliferations and the predominance of EBV latency type 2 even in polymorphic lesions are the main features of lymphoproliferations in patients with ITK deficiency, and suggest a unique pathomechanism of lymphomagenesis in this T-cell immunodeficiency.