Immunodeficiency-Related Lymphoid Proliferations: New Insights With Relevance to Practice
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MOLECULAR TESTING AND DIAGNOSTICS (J KHOURY, SECTION EDITOR)
Immunodeficiency-Related Lymphoid Proliferations: New Insights With Relevance to Practice Tapan Bhavsar 1 & Genevieve M. Crane 2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Our understanding of risk factors and mechanisms underlying immunosuppression-related lymphoproliferative disorders continues to evolve. An increasing number of patients are living with altered immune status due to HIV, solid organ or hematopoietic stem cell transplant, treatment of autoimmune disease, or advanced age. This review covers advances in understanding, emerging trends, and revisions to diagnostic guidelines. Recent Findings The tumor microenvironment, including interactions between the host immune system and tumor cells, is of increasing interest in the setting of immunosuppression. While some forms of lymphoproliferative disease are associated with unique risk factors, common mechanisms are also emerging. Indolent forms, such as Epstein-Barr virus positive mucocutaneous ulcer, are important to recognize. As methods to modulate the immune system evolve, more data are needed to understand and minimize lymphoproliferative disease risk. Summary A better understanding of individual risk factors and common mechanisms underlying immunosuppression-related lymphoproliferations will ultimately enable improved prevention and treatment of these disorders. Keywords Post-transplant lymphoproliferative disorder (PTLD) . Epstein-Barr virus (EBV) . Iatrogenic-associated lymphoproliferative disease . Lymphoma . HIV . EBV-positive mucocutaneous ulcer
Introduction In this brief review, we aim to highlight emerging areas of interest in immunosuppression-related lymphoproliferative disease (LPD), recent advances in our understanding of these disorders, and changes to diagnostic categories of direct relevance to patient care. In particular, while immunosuppressionrelated LPD is typically aggressive in nature, a subset is more indolent. These are important to differentiate as they may show a self-limited course or respond to reductions in immunosuppression alone (Table 1).
The World Health Organization (WHO) recognizes four major backgrounds in which immunosuppression-related LPD may arise (post-transplant, other iatrogenic therapy, HIV, and primary immunodeficiency). Chemotherapyrelated immune compromise and age-related immune senescence have also been associated with LPD risk [1•, 2–4]. While distinct risk factors for specific subtypes of LPD within these categories are emerging (Table 2), common mechanisms may also be of utility in understanding these disorders [13••, 24, 34•, 35••]. To aid in this, additional work is needed to better standardize nomenclature and diagnostic criteria across immunodeficiency settings to improve our ability to track and understand these disorders [36•].
This article is part of the Topical Collection on Molecular Testing and Diagnostics * Genevieve M. Crane [email protected] 1
Department of Pathology and Labo
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