Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?

Yasodha Natkunam, Dita Gratzinger, Amy Chadburn, John R. Goodlad, John K. C. Chan, Jonathan Said, Elaine S. Jaffe, Daphne de Jong

Research output: Contribution to journalReview articleAcademicpeer-review

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.
Original languageEnglish
Pages (from-to)1871-1878
JournalBlood
Volume132
Issue number18
DOIs
Publication statusPublished - 2018

Cite this

Natkunam, Y., Gratzinger, D., Chadburn, A., Goodlad, J. R., Chan, J. K. C., Said, J., ... de Jong, D. (2018). Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal? Blood, 132(18), 1871-1878. https://doi.org/10.1182/blood-2018-04-842559
Natkunam, Yasodha ; Gratzinger, Dita ; Chadburn, Amy ; Goodlad, John R. ; Chan, John K. C. ; Said, Jonathan ; Jaffe, Elaine S. ; de Jong, Daphne. / Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?. In: Blood. 2018 ; Vol. 132, No. 18. pp. 1871-1878.
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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. 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.",
author = "Yasodha Natkunam and Dita Gratzinger and Amy Chadburn and Goodlad, {John R.} and Chan, {John K. C.} and Jonathan Said and Jaffe, {Elaine S.} and {de Jong}, Daphne",
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Natkunam, Y, Gratzinger, D, Chadburn, A, Goodlad, JR, Chan, JKC, Said, J, Jaffe, ES & de Jong, D 2018, 'Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?' Blood, vol. 132, no. 18, pp. 1871-1878. https://doi.org/10.1182/blood-2018-04-842559

Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal? / Natkunam, Yasodha; Gratzinger, Dita; Chadburn, Amy; Goodlad, John R.; Chan, John K. C.; Said, Jonathan; Jaffe, Elaine S.; de Jong, Daphne.

In: Blood, Vol. 132, No. 18, 2018, p. 1871-1878.

Research output: Contribution to journalReview articleAcademicpeer-review

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T1 - Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal?

AU - Natkunam, Yasodha

AU - Gratzinger, Dita

AU - Chadburn, Amy

AU - Goodlad, John R.

AU - Chan, John K. C.

AU - Said, Jonathan

AU - Jaffe, Elaine S.

AU - de Jong, Daphne

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AB - 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.

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DO - 10.1182/blood-2018-04-842559

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Natkunam Y, Gratzinger D, Chadburn A, Goodlad JR, Chan JKC, Said J et al. Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal? Blood. 2018;132(18):1871-1878. https://doi.org/10.1182/blood-2018-04-842559