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Hematopoietic stem cell transplantation for progressive combined immunodeficiency and lymphoproliferation in patients with activated phosphatidylinositol-3-OH kinase δ syndrome type 1. | LitMetric

AI Article Synopsis

  • Activated phosphatidylinositol-3-OH kinase δ syndrome type 1 (APDS1) is an immunodeficiency disorder linked to mutations in the PIK3CD gene, leading to severe infections and lymphoid issues, and treatment through hematopoietic stem cell transplantation (HSCT) is being explored.* -
  • In a study of HSCT outcomes for APDS1 patients, the overall survival rate was 86.1%, but the rate of surviving without significant health events was only 39.6%, with many facing major complications despite improvements in symptoms post-transplant.* -
  • The research indicates that while HSCT can effectively address severe immunodeficiency and complications from APDS1, the

Article Abstract

Background: Activated phosphatidylinositol-3-OH kinase δ syndrome type 1 (APDS1) is a recently described primary immunodeficiency syndrome characterized by recurrent respiratory tract infections, lymphoid hyperplasia, and Herpesviridae infections caused by germline gain-of-function mutations of PIK3CD. Hematopoietic stem cell transplantation (HSCT) can be considered to ameliorate progressive immunodeficiency and associated malignancy, but appropriate indications, methods, and outcomes of HSCT for APDS1 remain undefined.

Objective: Our objective was to analyze the clinical manifestations, laboratory findings, prognosis, and treatment of APDS1 and explore appropriate indications and methods of HSCT.

Methods: We reviewed retrospectively the medical records of cohorts undergoing HSCT at collaborating facilities.

Results: Thirty-year overall survival was 86.1%, but event-free survival was 39.6%. Life-threatening events, such as severe infections or lymphoproliferation, were frequent in childhood and adolescence and were common indications for HSCT. Nine patients underwent HSCT with fludarabine-based reduced-intensity conditioning. Seven patients survived after frequent adverse complications and engraftment failure. Most symptoms improved after HSCT.

Conclusion: Patients with APDS1 showed variable clinical manifestations. Life-threatening progressive combined immunodeficiency and massive lymphoproliferation were common indications for HSCT. Fludarabine-based reduced-intensity conditioning-HSCT ameliorated clinical symptoms, but transplantation-related complications were frequent, including graft failure.

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Source
http://dx.doi.org/10.1016/j.jaci.2018.04.032DOI Listing

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