Sustained and full fetal hemoglobin production after failure of bone marrow transplant in a patient homozygous for beta 0-thalassemia: a clinical remission despite genetic disease and transplant rejection

Am J Hematol. 2009 Jun;84(6):372-3. doi: 10.1002/ajh.21392.

Abstract

An adult patient affected by beta(0)-thalassemia major underwent allogeneic bone marrow transplant (BMT) from a matched related donor. Forty days after transplant, allogeneic engraftment failure and autologous beta(0)-thalassemic bone marrow recovery were documented. Red blood cell transfusions were required until 118 days post-transplant. Thereafter, the haemoglobin (Hb) levels stabilized over 11.8 gr/dl throughout the ongoing 34-month follow-up, abolishing the need for transfusion support. The Hb electrophoresis showed 100% Hb Fetal (HbF). This unexplained case suggests full HbF production may occur in an adult patient with beta(0)-thalassemia major.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Adolescent
  • Bone Marrow Transplantation*
  • Fetal Hemoglobin / biosynthesis*
  • Graft Rejection / blood*
  • Humans
  • beta-Thalassemia / blood*
  • beta-Thalassemia / genetics
  • beta-Thalassemia / surgery

Substances

  • Fetal Hemoglobin