Premium
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
Author(s) -
Paciaroni Katia,
Gallucci Cristiano,
De Angelis Gioia,
Alfieri Cecilia,
Roveda Andrea,
Lucarelli Guido
Publication year - 2009
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21392
Subject(s) - medicine , thalassemia , bone marrow transplant , hemoglobinopathy , bone marrow , fetal hemoglobin , beta thalassemia , fetus , bone marrow transplantation , disease , gastroenterology , surgery , pregnancy , biology , genetics
An adult patient affected by β 0 ‐thalassemia major underwent allogeneic bone marrow transplant (BMT) from a matched related donor. Forty days after transplant, allogeneic engraftment failure and autologous β 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 β 0 ‐thalassemia major. Am. J. Hematol. 2009. © 2009 Wiley‐Liss, Inc.