Carlos Murga-Zamalloa, Michael Brandon Stone, Marc G Gutierrez, Neha Rajendra Hippalgaonkar, Hamza Tariq, Morteza Sadeh, Ankit Mehta, Irum Khan, Serhan Alkan, Kedar V Inamdar, Ryan Wilcox, Amir Behdad
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A small subset of immunocompetent patients develops CAEBV, a life-threatening complication resulting from EBV-infected T-cell or NK cell neoplastic lymphocytes. The sites of end-organ damage in T/NK sCAEBV demonstrate pathologic findings such as reactive lymphoid proliferations, making the diagnosis difficult to establish, with the only curative option being an allogeneic hematopoietic stem cell transplant.</p><p><strong>Conclusions: </strong>This diagnosis is most prevalent in Asia, with few cases reported in Western countries. Adult age is an independent risk factor for poor outcomes, and most cases are diagnosed in pediatric populations.</p>","PeriodicalId":7506,"journal":{"name":"American journal of clinical pathology","volume":null,"pages":null},"PeriodicalIF":2.3000,"publicationDate":"2024-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Characterization of T-/natural killer cell lymphoproliferative neoplasms associated with systemic, chronic, active Epstein-Barr virus in adults: A report of 5 cases in a Western population.\",\"authors\":\"Carlos Murga-Zamalloa, Michael Brandon Stone, Marc G Gutierrez, Neha Rajendra Hippalgaonkar, Hamza Tariq, Morteza Sadeh, Ankit Mehta, Irum Khan, Serhan Alkan, Kedar V Inamdar, Ryan Wilcox, Amir Behdad\",\"doi\":\"10.1093/ajcp/aqad184\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objectives: </strong>Because of its low frequency in adult populations and clinical and laboratory overlap with hemophagocytic lymphohistiocytosis and other T-cell lymphomas, T-cell/natural killer (NK) cell systemic, chronic, active Epstein-Barr virus (EBV) (T/NK sCAEBV) infection remains underdiagnosed, preventing critical, prompt therapeutic interventions.</p><p><strong>Methods: </strong>We report a 5-case series that included 2 adult patients with T/NK sCAEBV and 3 additional adult patients with T/NK lymphomas with concomitant systemic EBV infection to review these entities' overlapping diagnostic and clinical features.</p><p><strong>Results: </strong>Approximately 95% of the world population has been infected with EBV during their lifetime, and infection is usually asymptomatic, with symptomatic cases eventually resolving spontaneously. A small subset of immunocompetent patients develops CAEBV, a life-threatening complication resulting from EBV-infected T-cell or NK cell neoplastic lymphocytes. The sites of end-organ damage in T/NK sCAEBV demonstrate pathologic findings such as reactive lymphoid proliferations, making the diagnosis difficult to establish, with the only curative option being an allogeneic hematopoietic stem cell transplant.</p><p><strong>Conclusions: </strong>This diagnosis is most prevalent in Asia, with few cases reported in Western countries. 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引用次数: 0
摘要
研究目的由于T细胞/自然杀伤(NK)细胞全身性、慢性、活动性爱泼斯坦-巴氏病毒(EBV)(T/NK sCAEBV)感染在成人人群中发病率低,且与嗜血细胞淋巴组织细胞增多症和其他T细胞淋巴瘤在临床和实验室方面存在重叠,因此该病仍未得到充分诊断,导致无法及时采取关键的治疗措施:我们报告了5例系列病例,其中包括2例T/NK sCAEBV成人患者和另外3例T/NK淋巴瘤合并全身EBV感染的成人患者,以回顾这些实体重叠的诊断和临床特征:全球约 95% 的人在一生中感染过 EBV,感染后通常无症状,有症状的病例最终会自愈。一小部分免疫功能正常的患者会罹患 CAEBV,这是一种由受 EBV 感染的 T 细胞或 NK 细胞肿瘤性淋巴细胞引起的危及生命的并发症。T/NK sCAEBV的终末器官损伤部位会出现反应性淋巴细胞增生等病理表现,因此很难确诊,唯一的治疗方案是异体造血干细胞移植:结论:这种诊断在亚洲最为普遍,西方国家报告的病例很少。成人年龄是导致不良后果的一个独立风险因素,大多数病例在儿童群体中确诊。
Characterization of T-/natural killer cell lymphoproliferative neoplasms associated with systemic, chronic, active Epstein-Barr virus in adults: A report of 5 cases in a Western population.
Objectives: Because of its low frequency in adult populations and clinical and laboratory overlap with hemophagocytic lymphohistiocytosis and other T-cell lymphomas, T-cell/natural killer (NK) cell systemic, chronic, active Epstein-Barr virus (EBV) (T/NK sCAEBV) infection remains underdiagnosed, preventing critical, prompt therapeutic interventions.
Methods: We report a 5-case series that included 2 adult patients with T/NK sCAEBV and 3 additional adult patients with T/NK lymphomas with concomitant systemic EBV infection to review these entities' overlapping diagnostic and clinical features.
Results: Approximately 95% of the world population has been infected with EBV during their lifetime, and infection is usually asymptomatic, with symptomatic cases eventually resolving spontaneously. A small subset of immunocompetent patients develops CAEBV, a life-threatening complication resulting from EBV-infected T-cell or NK cell neoplastic lymphocytes. The sites of end-organ damage in T/NK sCAEBV demonstrate pathologic findings such as reactive lymphoid proliferations, making the diagnosis difficult to establish, with the only curative option being an allogeneic hematopoietic stem cell transplant.
Conclusions: This diagnosis is most prevalent in Asia, with few cases reported in Western countries. Adult age is an independent risk factor for poor outcomes, and most cases are diagnosed in pediatric populations.
期刊介绍:
The American Journal of Clinical Pathology (AJCP) is the official journal of the American Society for Clinical Pathology and the Academy of Clinical Laboratory Physicians and Scientists. It is a leading international journal for publication of articles concerning novel anatomic pathology and laboratory medicine observations on human disease. AJCP emphasizes articles that focus on the application of evolving technologies for the diagnosis and characterization of diseases and conditions, as well as those that have a direct link toward improving patient care.