. 2022 Jun 22;36(7):1720–1748. doi: 10.1038/s41375-022-01620-2

Table 5.

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

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

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