Similar outcome of upfront-unrelated and matched sibling stem cell transplantation in idiopathic paediatric aplastic anaemia. A study on behalf of the UK Paediatric BMT Working Party, Paediatric Diseases Working Party and Severe Aplastic Anaemia Working Party of EBMT

Br J Haematol. 2015 Nov;171(4):585-94. doi: 10.1111/bjh.13614. Epub 2015 Jul 28.

Abstract

We explored the feasibility of unrelated donor haematopoietic stem cell transplant (HSCT) upfront without prior immunosuppressive therapy (IST) in paediatric idiopathic severe aplastic anaemia (SAA). This cohort was then compared to matched historical controls who had undergone first-line therapy with a matched sibling/family donor (MSD) HSCT (n = 87) or IST with horse antithymocyte globulin and ciclosporin (n = 58) or second-line therapy with unrelated donor HSCT post-failed IST (n = 24). The 2-year overall survival in the upfront cohort was 96 ± 4% compared to 91 ± 3% in the MSD controls (P = 0·30) and 94 ± 3% in the IST controls (P = 0·68) and 74 ± 9% in the unrelated donor HSCT post-IST failure controls (P = 0·02).The 2-year event-free survival in the upfront cohort was 92 ± 5% compared to 87 ± 4% in MSD controls (P = 0·37), 40 ± 7% in IST controls (P = 0·0001) and 74 ± 9% in the unrelated donor HSCT post-IST failure controls (n = 24) (P = 0·02). Outcomes for upfront-unrelated donor HSCT in paediatric idiopathic SAA were similar to MSD HSCT and superior to IST and unrelated donor HSCT post-IST failure. Front-line therapy with matched unrelated donor HSCT is a novel treatment approach and could be considered as first-line therapy in selected paediatric patients who lack a MSD.

Keywords: aplastic anaemia; paediatric aplastic anaemia; transplantation.

Publication types

  • Comparative Study

MeSH terms

  • Adenoviridae Infections / drug therapy
  • Adenoviridae Infections / epidemiology
  • Adolescent
  • Adult
  • Anemia, Aplastic / mortality
  • Anemia, Aplastic / therapy*
  • Antilymphocyte Serum
  • Blood Transfusion / statistics & numerical data
  • Bone Marrow Transplantation / adverse effects
  • Bone Marrow Transplantation / statistics & numerical data*
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Cyclosporine / therapeutic use
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Graft vs Host Disease / drug therapy
  • Graft vs Host Disease / epidemiology
  • Graft vs Host Disease / prevention & control
  • Herpesviridae Infections / drug therapy
  • Herpesviridae Infections / epidemiology
  • Histocompatibility
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Kaplan-Meier Estimate
  • Length of Stay / statistics & numerical data
  • Living Donors
  • Male
  • Peripheral Blood Stem Cell Transplantation / adverse effects
  • Peripheral Blood Stem Cell Transplantation / statistics & numerical data*
  • Postoperative Complications / drug therapy
  • Postoperative Complications / epidemiology
  • Primary Graft Dysfunction / epidemiology
  • Quality of Life
  • Retrospective Studies
  • Siblings
  • Survival Rate
  • T-Lymphocytes
  • Treatment Outcome
  • Virus Activation
  • Young Adult

Substances

  • Antilymphocyte Serum
  • Immunosuppressive Agents
  • Cyclosporine