Pediatric Plasmablastic Lymphoma in the Setting of CD70 Deficiency

Baskin K., VURAL Ö., HASKOLOĞLU Z. Ş., ERMAN B., UYAR GÖÇÜN F. P., OKUR A., ...More

Journal of Pediatric Hematology/Oncology, vol.46, no.8, pp.438-441, 2024 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 46 Issue: 8
  • Publication Date: 2024
  • Doi Number: 10.1097/mph.0000000000002948
  • Journal Name: Journal of Pediatric Hematology/Oncology
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, MEDLINE
  • Page Numbers: pp.438-441
  • Keywords: CD70 deficiency, pediatric, plasmablastic lymphoma, primary immunodeficiency
  • Gazi University Affiliated: Yes

Abstract

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.