A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)

Conclusions
Though this is not a comprehensive summary of malignancies in PIDDs, or even lymphoproliferative disease in this area, this review summarizes the Medline-indexed published reports of B and T lymphomas in patients with PIDDs. This report highlights the diversity of malignant lymphoproliferative disorders in setting of PIDDs, and its associated challenges of diagnosis and treatment. The pathological classification and nomenclature for the lymphoid malignancies with variably reported and postulated underlying mechanisms were inconsistent and inadequate for many of these published reports. A wide range of treatment options were utilized, and response rate was highly variable suggesting an empirical approach rather than a systematic and tailored treatment regimen, based on underlying genetic defect, and degree of immunological impairment. HCT and gene therapy (where available) remains the best treatment option for many, but not all of these patients, and should be promptly initiated after diagnosis, particularly in some conditions, such as SCID, WAS, IL10 receptor deficiency among others. HCT, as a therapeutic option, remains significantly under-utilized in adult patients, likely related to inadequate awareness among adult hematologists, and these patients may benefit from increased utilization of HCT in appropriate settings. We highlight the significant need of unifying nomenclature, pathological analysis, and assessment of mechanisms of lymphomagenesis in these pati...

Source: Frontiers in Immunology - April 15, 2019 Category: Allergy & Immunology Source Type: research