Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?

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Abstract

Immunodeficiency-associated lymphoproliferative disorders (IA-LPDs) are pathologically and clinically heterogeneous. In many instances, similar features are shared by a spectrum of IA-LPDs in clinically diverse settings. The World Health Organization (WHO), however, classifies IA-LPDs by their immunodeficiency setting largely according to the paradigm of post-transplant lymphoproliferative disorders (PTLD), but with inconsistent terminology and disease definitions. The field currently lacks standardization and would greatly benefit from thinking across immunodeficiency categories by adopting a common working vocabulary to better understand these disorders and guide clinical management. We propose a three-part unifying nomenclature that includes the name of the lesion, associated virus and the specific immunodeficiency setting for all IA-LPDs. B-cell LPDs are usually EBV-positive and show a spectrum of lesions including hyperplasias, polymorphic LPDs, aggressive lymphomas and, rarely, indolent lymphomas. HHV8-associated LPDs also include both polyclonal and monoclonal proliferations. EBV-negative B-cell LPDs and T- and NK-cell LPDs are rare and less well characterized. Recognition of any immunodeficiency is important as it impacts the choice of treatment options. There is an urgent need for reappraisal of IA-LPDs because a common framework will facilitate meaningful biological insights and pave the way for future work in the field.

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