Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?

TitleImmunodeficiency-associated lymphoproliferative disorders: time for reappraisal?
Publication TypeJournal Article
Year of Publication2018
AuthorsNatkunam Y, Gratzinger D, Chadburn A, Goodlad JR, Chan JKC, Said J, Jaffe ES, de Jong D
JournalBlood
Volume132
Issue18
Pagination1871-1878
Date Published2018 11 01
ISSN1528-0020
KeywordsEpstein-Barr Virus Infections, Herpesviridae Infections, Herpesvirus 4, Human, Herpesvirus 8, Human, Humans, Immunologic Deficiency Syndromes, Killer Cells, Natural, Lymphoproliferative Disorders, T-Lymphocytes
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. However, the World Health Organization (WHO) classifies IA-LPDs by their immunodeficiency setting largely according to the paradigm of posttransplant lymphoproliferative disorders 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 3-part unifying nomenclature that includes the name of the lesion, associated virus, and the specific immunodeficiency setting for all IA-LPDs. B-cell lymphoproliferative disorders (LPDs) are usually Epstein-Barr virus (EBV) and show a spectrum of lesions, including hyperplasias, polymorphic LPDs, aggressive lymphomas, and, rarely, indolent lymphomas. Human herpes virus 8-associated LPDs also include polyclonal and monoclonal proliferations. EBV B-cell LPDs and T- and NK-cell LPDs are rare and less well characterized. Recognition of any immunodeficiency is important because 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.

DOI10.1182/blood-2018-04-842559
Alternate JournalBlood
PubMed ID30082493
PubMed Central IDPMC6213318
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