Immune deficiency/dysregulation -associated lymphoproliferative disorders. Revised classification and management

Abstract

Significant advances in the field of lymphoma have resulted in two recent classification proposals, the International Consensus Classification (ICC) and the 5th edition WHO. A few entities are categorized differently in the ICC compared to the WHO. Nowhere is this more apparent than the immunodeficiency lymphoproliferative disorders. The three previous versions of the WHO classification (3rd, 4th and revised 4th editions) and the ICC focused on four clinical settings in which these lesions arise for primary categorization. In contrast the 2023 WHO 5th edition includes pathologic characteristics including morphology and viral status, in addition to clinical setting, as important information for lesion classification. In addition, the 2023 WHO recognizes a broader number of clinical scenarios in which these lesions arise, including not only traditional types of immune deficiency but also immune dysregulation. With this classification it is hoped that new treatment strategies will be developed leading to better patient outcomes.

Keywords: Classification; EBV; Immune deficiency-associated lymphomas; Immune deficiency-associated lymphoproliferative disorders; Immune deficiency/dysregulation setting; Immune dysregulation-associated lymphoproliferative disorders; KSHV/HHV8; Management.