Paroxysmal nocturnal hemoglobinuria and telomere length predicts response to immunosuppressive therapy in pediatric aplastic anemia

Haematologica
Atsushi NaritaJapan Childhood Aplastic Anemia Study Group

Abstract

Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length were identified as predictive biomarkers of immunosuppressive therapy responsiveness in aplastic anemia. We enrolled 113 aplastic anemia patients (63 boys and 50 girls) in this study to evaluate their response to immunosuppressive therapy. The paroxysmal nocturnal hemoglobinuria populations and telomere length were detected by flow cytometry. Forty-seven patients (42%) carried a minor paroxysmal nocturnal hemoglobinuria population. The median telomere length of aplastic anemia patients was -0.99 standard deviation (SD) (range -4.01-+3.01 SD). Overall, 60 patients (53%) responded to immunosuppressive therapy after six months. Multivariate logistic regression analysis identified the absence of a paroxysmal nocturnal hemoglobinuria population and a shorter telomere length as independent unfavorable predictors of immunosuppressive therapy response at six months. The cohort was stratified into a group of poor prognosis (paroxysmal nocturnal hemoglobinuria negative and shorter telome...Continue Reading

Citations

Apr 20, 2016·International Journal of Hematology·Atsushi Narita, Seiji Kojima
Jun 10, 2016·International Journal of Hematology·Andrea BacigalupoSimona Sica
Sep 7, 2016·Cytometry. Part B, Clinical Cytometry·Marta MoradoUNKNOWN PNH working group of the Iberian Society of Cytometry (SIC)
Jan 21, 2017·British Journal of Haematology·Natasha StanleyDaria V Babushok
Jan 18, 2017·Blood·Andrea Bacigalupo
Oct 27, 2017·Journal of Pediatric Hematology/oncology·Shuanglong LuXiaotian Xie
Nov 17, 2017·Current Treatment Options in Oncology·Scott A PeslakDaria V Babushok
Jun 24, 2017·British Journal of Haematology·Atsushi NaritaSeiji Kojima
Aug 16, 2018·Blood Advances·George E GeorgesRainer Storb
Sep 7, 2017·Current Opinion in Hematology·Rajat KumarBiju George
May 24, 2020·Bone Marrow Transplantation·Nao YoshidaUNKNOWN Pediatric Aplastic Anemia Working Group of the Japan Society for Hematopoietic Cell Transplantation
Apr 10, 2020·Stem Cell Reviews and Reports·Katarzyna Brzeźniakiewicz-JanusLidia Gil
Dec 4, 2016·Hematology·Danielle M Townsley, Thomas Winkler
Oct 26, 2018·The New England Journal of Medicine·Neal S Young
Jan 5, 2021·Molecular Biology Reports·Anjali ShahBabu Rao Vundinti
Apr 16, 2021·British Journal of Haematology·Phillip Scheinberg

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