Literature DB >> 19373892

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.

Katia Paciaroni, Cristiano Gallucci, Gioia De Angelis, Cecilia Alfieri, Andrea Roveda, Guido Lucarelli.   

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.

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Year:  2009        PMID: 19373892     DOI: 10.1002/ajh.21392

Source DB:  PubMed          Journal:  Am J Hematol        ISSN: 0361-8609            Impact factor:   10.047


  4 in total

1.  Transfusion-independent β(0)-thalassemia after bone marrow transplantation failure: proposed involvement of high parental HbF and an epigenetic mechanism.

Authors:  Katia Paciaroni; Guido Lucarelli; Fabrizio Martelli; Anna Rita Migliaccio; Marieke von Lindern; Joseph Borg; Nynke Gillemans; Thamar B van Dijk; Sjaak Philipsen
Journal:  Am J Blood Res       Date:  2014-09-05

2.  Structural and Functional Insights on an Uncharacterized Aγ-Globin-Gene Polymorphism Present in Four β0-Thalassemia Families with High Fetal Hemoglobin Levels.

Authors:  Nicoletta Bianchi; Lucia Carmela Cosenza; Ilaria Lampronti; Alessia Finotti; Giulia Breveglieri; Cristina Zuccato; Enrica Fabbri; Giovanni Marzaro; Adriana Chilin; Gioia De Angelis; Monica Borgatti; Cristiano Gallucci; Cecilia Alfieri; Michela Ribersani; Antonella Isgrò; Marco Marziali; Javid Gaziev; Aldo Morrone; Pietro Sodani; Guido Lucarelli; Roberto Gambari; Katia Paciaroni
Journal:  Mol Diagn Ther       Date:  2016-04       Impact factor: 4.074

Review 3.  Hematopoietic stem cell transplantation in thalassemia and sickle cell anemia.

Authors:  Guido Lucarelli; Antonella Isgrò; Pietro Sodani; Javid Gaziev
Journal:  Cold Spring Harb Perspect Med       Date:  2012-05       Impact factor: 6.915

4.  Family cord blood banking for sickle cell disease: a twenty-year experience in two dedicated public cord blood banks.

Authors:  Hanadi Rafii; Françoise Bernaudin; Helene Rouard; Valérie Vanneaux; Annalisa Ruggeri; Marina Cavazzana; Valerie Gauthereau; Aurélie Stanislas; Malika Benkerrou; Mariane De Montalembert; Christele Ferry; Robert Girot; Cecile Arnaud; Annie Kamdem; Joelle Gour; Claudine Touboul; Audrey Cras; Mathieu Kuentz; Claire Rieux; Fernanda Volt; Barbara Cappelli; Karina T Maio; Annalisa Paviglianiti; Chantal Kenzey; Jerome Larghero; Eliane Gluckman
Journal:  Haematologica       Date:  2017-03-16       Impact factor: 9.941

  4 in total

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