Table 5 Three-part nomenclature for lymphoid proliferations and lymphomas arising in the setting of immune deficiency/dysregulation.

From: The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms

Histological diagnosis

Viral association

Immune deficiency/dysregulation setting

◦  Hyperplasia (specify type)

◦  Polymorphic lymphoproliferative disorder

◦  Mucocutaneous ulcer

◦  Lymphoma (classify as for immunocompetent patients)

◦  EBV +/−

◦  KSHV/HHV8 +/−

◦  Inborn error of immunity (specify type)

◦  HIV infection

◦  Posttransplant (specify: solid organ/bone marrow)

◦  Autoimmune disease

◦  Iatrogenic/therapy-related (specify)

◦  Immune senescence

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