Long-term outcome of acquired aplastic anaemia in children: comparison between immunosuppressive therapy and bone marrow transplantation

Br J Haematol. 2000 Oct;111(1):321-8. doi: 10.1046/j.1365-2141.2000.02289.x.

Abstract

A total of 100 children under the age of 17 years with acquired aplastic anaemia (AA) were initially treated with immunosuppressive therapy (IST) (n = 63) or bone marrow transplantation (BMT) (n = 37) from an HLA-matched family donor. The projected 10-year survival rates were 55 +/- 8% and 97 +/- 3% respectively (P = 0.004). Because the IST group included 11 non-responders who were salvaged by BMT from an HLA-matched unrelated donor, we compared failure-free survival (FFS) between the groups. The probability of FFS at 10 years was 97 +/- 3% for the BMT group, compared with 40 +/- 8% for the IST group (P = 0.0001). Seven patients evolved to myelodysplastic syndrome (MDS) with monosomy 7 and the estimated cumulative incidence of MDS 10 years after diagnosis was 20 +/- 7% in the IST group. We compared the outcome of children treated with IST during the two consecutive periods of 1983-91 (group A, n = 40) and 1991-8 (group B, n = 23) to assess the impact of combined therapy with antithymocyte globulin and cyclosporin. The probability of FFS at 7 years follow-up was the same in the two groups (50 +/- 8% vs. 40 +/- 15%, P = 0.40). We recommend BMT as first-line therapy in paediatric severe AA patients with an HLA-matched family donor. Alternative donor BMT is recommended as salvage therapy in patients who relapse or do not respond to initial IST.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Anemia, Aplastic / mortality
  • Anemia, Aplastic / surgery
  • Anemia, Aplastic / therapy*
  • Antilymphocyte Serum / therapeutic use*
  • Bone Marrow Transplantation*
  • Cell Count
  • Child
  • Child, Preschool
  • Cyclosporine / therapeutic use*
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Graft vs Host Disease / complications
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Male
  • Retrospective Studies
  • Statistics, Nonparametric
  • T-Lymphocytes / immunology*

Substances

  • Antilymphocyte Serum
  • Immunosuppressive Agents
  • Cyclosporine