Shahid Sanam, Rossi Christopher, Vyas Pranav, Bollard Catherine, Dave Hema
Children's National Health System , Washington , D.C.
Pediatr Hematol Oncol. 2019 Aug;36(5):309-316. doi: 10.1080/08880018.2019.1637982. Epub 2019 Jul 17.
Iatrogenic immunodeficiency-associated lymphoproliferative disorders (LPDs) are a group of lymphoid proliferations or lymphomas that are well known to be associated with an immunosuppressed state. These disorders most commonly occur following hematopoietic or solid organ transplantation (called post-transplant lymphoproliferative disorders or PTLD), but cases have also been described during the treatment of autoimmune and rheumatologic disorders by immunosuppressive and immunomodulatory medications. These disorders are strongly associated with infection by the Epstein-Barr virus (EBV) as a result of impaired immune function in the immunosuppressed state. While this phenomenon has been well documented in autoimmune conditions, cases affecting pediatric patients while on anti-leukemia chemotherapy are lacking. In this report, we describe a case of a pediatric immunosuppressed patient with recurrent sinusitis found to have a nasopharyngeal mass consistent with EBV-positive B-cell lymphoproliferative disorder resembling a polymorphic PTLD during the maintenance phase of B-cell Acute Lymphoblastic Leukemia (ALL) therapy. The patient was successfully treated with rituximab without any cytotoxic chemotherapy, highlighting the importance of recognizing this clinical entity in non-transplant patients with hematologic malignancies.
医源性免疫缺陷相关淋巴增殖性疾病（LPDs）是一组已知与免疫抑制状态相关的淋巴样增殖或淋巴瘤。这些疾病最常发生在造血或实体器官移植后（称为移植后淋巴增殖性疾病或PTLD），但也有病例在通过免疫抑制和免疫调节药物治疗自身免疫性和风湿性疾病期间被描述。由于免疫抑制状态下免疫功能受损，这些疾病与爱泼斯坦-巴尔病毒（EBV）感染密切相关。虽然这种现象在自身免疫性疾病中已有充分记录，但缺乏在接受抗白血病化疗的儿科患者中发生的病例。在本报告中，我们描述了一例儿科免疫抑制患者，该患者在B细胞急性淋巴细胞白血病（ALL）维持治疗阶段反复出现鼻窦炎，发现鼻咽部肿块，符合EBV阳性B细胞淋巴增殖性疾病，类似于多形性PTLD。该患者在未进行任何细胞毒性化疗的情况下成功接受了利妥昔单抗治疗，突出了在患有血液系统恶性肿瘤的非移植患者中识别这一临床实体的重要性。
