ALK-positive, EBV-positive Large B-cell lymphoma in an HIV patient: a diagnostic pitfall mimicking plasmablastic lymphoma.

IF 0.6 4区医学 Q4 HEMATOLOGY

Journal of Hematopathology Pub Date : 2026-03-10 DOI:10.1007/s12308-026-00688-4

Chutima Pinnark Surintrspanont, Benjamaporn Buasatit, Narittee Sukswai, Suphakit Hemla, Chinachote Teerapakpinyo, Shanop Shuangshoti

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引用次数: 0

Abstract

ALK-positive large B-cell lymphoma (ALK + LBCL) is a rare subtype of large B-cell lymphoma characterized by plasmablastic morphology, intra-sinusoidal or sheet-like proliferation, loss of pan-B-cell markers, and expression of plasmacytic markers. According to WHO classification, CD30 expression is generally absent and Epstein-Barr virus (EBV) infection is not detected [1,2,5] . We aim to report the first case of ALK-positive, EBV-positive large B-cell lymphoma (ALK + EBV + LBCL) in an HIV-infected patient. A 60-year-old Thai male with HIV infection and severe immunosuppression (CD4 count: 93 cells/mm³) presented with generalized lymphadenopathy and an epiglottic mass. Biopsy showed a plasmablastic neoplasm proliferating in loose sheets, negative for pan-B-cell markers except OCT2, and positive for plasmacytic markers and CD30. The tumor also expressed EBER, ALK1 (paranuclear dot pattern), and ALK D5F3; HHV8 was negative. FISH confirmed ALK gene rearrangement, and targeted sequencing revealed a GORASP2::ALK fusion gene. The diagnosis of ALK + EBV + LBCL in the setting of HIV-related immunodeficiency was established. The patient developed pneumonia with septic shock and died during admission. EBV-positive plasmablastic B-cell neoplasms in HIV-infected patients are not always plasmablastic lymphoma. Immunohistochemistry for ALK and HHV8 should be included in the diagnostic panel. Importantly, CD30 and EBER positivity do not exclude ALK + LBCL.

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alk阳性，ebv阳性的HIV患者的大b细胞淋巴瘤：一个模拟浆母细胞淋巴瘤的诊断陷阱。

ALK阳性大b细胞淋巴瘤（ALK + LBCL）是一种罕见的大b细胞淋巴瘤亚型，其特征是浆母细胞形态，窦内或片状增生，泛b细胞标志物缺失，浆细胞标志物表达。根据WHO的分类，CD30一般不表达，未检测到eb病毒（Epstein-Barr virus， EBV）感染[1,2,5]。我们的目标是报告第一例ALK阳性，EBV阳性大b细胞淋巴瘤（ALK + EBV + LBCL）在hiv感染患者。一名60岁泰国男性HIV感染和严重免疫抑制（CD4计数：93细胞/mm3），表现为全身性淋巴结病和会阴肿块。活检显示浆母细胞肿瘤呈松散片状增生，除OCT2外，泛b细胞标志物呈阴性，浆母细胞标志物和CD30呈阳性。肿瘤还表达了EBER、ALK1（核旁点状图）和ALK D5F3；HHV8阴性。FISH证实ALK基因重排，靶向测序显示一个GORASP2::ALK融合基因。建立了ALK + EBV + LBCL在hiv相关免疫缺陷背景下的诊断。患者出现肺炎并感染性休克，入院时死亡。hiv感染患者的ebv阳性浆母细胞b细胞肿瘤并不总是浆母细胞淋巴瘤。ALK和HHV8的免疫组化应包括在诊断小组中。重要的是，CD30和EBER阳性不排除ALK + LBCL。

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来源期刊

Journal of Hematopathology HEMATOLOGYPATHOLOGY-PATHOLOGY

CiteScore

0.80

自引率

0.00%

发文量

期刊介绍： The Journal of Hematopathology aims at providing pathologists with a special interest in hematopathology with all the information needed to perform modern pathology in evaluating lymphoid tissues and bone marrow. To this end the journal publishes reviews, editorials, comments, original papers, guidelines and protocols, papers on ancillary techniques, and occasional case reports in the fields of the pathology, molecular biology, and clinical features of diseases of the hematopoietic system. The journal is the unique reference point for all pathologists with an interest in hematopathology. Molecular biologists involved in the expanding field of molecular diagnostics and research on lymphomas and leukemia benefit from the journal, too. Furthermore, the journal is of major interest for hematologists dealing with patients suffering from lymphomas, leukemias, and other diseases. The journal is unique in its true international character. Especially in the field of hematopathology it is clear that there are huge geographical variations in incidence of diseases. This is not only locally relevant, but due to globalization, relevant for all those involved in the management of patients.