Lymphoid proliferations and lymphomas associated with immune deficiency

WHO 2022

Histologie + Virus + Immuun deficientie/dysregulatie = diagnose

WHO-HAEM5 has introduced major changes to the classification of immunodeficiency-associated lymphoproliferative disorders. The new standardized nomenclature builds on an integrated approach to diagnosis that combines all relevant data into a reporting system as follows;

  1. Histological diagnosis according to accepted criteria and terminology;

  2. Presence or absence of one or more oncogenic virus(es); and

  3. The clinical setting/immunodeficiency background.

IEI; inborn errors of immunity, EBVMCU: EBV-positive mucocutaneous ulcer.

Primary immunodeficiencies, associated with germline mutations, have been renamed “inborn errors of immunity” (IEI) by the International Union of Immunological Societies, a terminology adopted by WHO-HAEM5. Patients with IEI may develop distinctive types of lymphoid proliferations unique to the particular IEI, as well as those described in the acquired IDD settings. The types and frequency of these proliferations are largely dependent on the immune dysregulation conferred by the germline aberration underyling a respective IEI.