Department of Pediatric Allergy and Immunology.
Department of Pediatric Oncology.
J Pediatr Hematol Oncol. 2024 Nov 1;46(8):438-441. doi: 10.1097/MPH.0000000000002948. Epub 2024 Sep 5.
Combined immunodeficiency due to CD70 deficiency is characterized by increased susceptibility to infections, hypogammaglobulinemia, and malignancy. These patients typically present with chronic Epstein Barr virus (EBV) viremia, severe EBV-related hemophagocytic lymphohistiocytosis, lymphoproliferation, and Hodgkin and non-Hodgkin lymphomas. Plasmablastic lymphoma (PBL) is an extremely rare malignancy in all ages and is predominantly seen in male adults with human immunodeficiency virus infection. EBV infection, immunosuppression, solid organ transplantation, and age-related immune deterioration are also suspected causes of PBL. Nevertheless, there is scarce data about its association with primary immunodeficiencies in the literature. Here, we present the first case of a CD70 -deficient pediatric patient with PBL.
由于 CD70 缺乏导致的联合免疫缺陷的特征是易感染、低丙种球蛋白血症和恶性肿瘤。这些患者通常表现为慢性 EBV（Epstein Barr virus，EBV）病毒血症、严重的 EBV 相关噬血细胞性淋巴组织细胞增生症、淋巴组织增生以及霍奇金和非霍奇金淋巴瘤。浆母细胞淋巴瘤（plasmablastic lymphoma，PBL）在所有年龄段都是一种极其罕见的恶性肿瘤，主要发生在感染人类免疫缺陷病毒的成年男性中。EBV 感染、免疫抑制、实体器官移植和与年龄相关的免疫功能下降也是 PBL 的可疑病因。然而，文献中关于其与原发性免疫缺陷的关联的数据很少。在这里，我们报告了首例 CD70 缺陷的儿童患者伴发 PBL 的病例。
