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Bone marrow transplantation in severe Glanzmann's thrombasthenia with antiplatelet alloimmunization

Bone Marrow Transplantation volume 25, pages 327–330 (2000)Cite this article

Abstract

Glanzmann's thrombasthenia is an autosomal recessive disorder characterized by a lack of platelet aggregation due to the absence of platelet glycoprotein IIb and IIIa. Usually, the disease leads to mild hemorrhage but sometimes bleeding is severe enough to be life-threatening. We report the case of a 16-year-old girl, presenting with very severe type 1 Glanzmann's thrombasthenia, successfully treated with an HLA-identical sibling bone marrow transplant (BMT). We also update the clinical and laboratory data of her brother, who had received a BMT 16 years ago for the same disease. In the light of these two cases and two others published in the literature, we discuss the indications for BMT from HLA-identical sibling donors in Glanzmann's thrombasthenia. Alloimmunization against the missing platelet GPIIb/IIIa complex and severity of bleeding episodes may constitute sufficient criteria for allogeneic BMT after careful assessment of the risk-benefit of such a procedure, although this remains exceptional in this disease. Bone Marrow Transplantation (2000) 25, 327–330.

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Authors and Affiliations

  1. Laboratory of Hematology, Hôpital Lariboisière, France

    S Bellucci

  2. Bone Marrow Transplant Unit, Hôpital Saint Louis, Paris, France

    G Damaj, V Rocha, A Devergie, IYacoub Agha, L Garderet, P Ribaud, G Socié & E Gluckman

  3. Blood Bank AP/HP, Hôpital Lariboisière, France

    B Boval

  4. Blood Bank AP/HP, Hôpital Saint Louis, Paris, France

    R Traineau

Authors
  1. S Bellucci
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  2. G Damaj
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  3. B Boval
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  4. V Rocha
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  5. A Devergie
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  6. IYacoub Agha
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  7. L Garderet
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  8. P Ribaud
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  9. R Traineau
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  10. G Socié
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  11. E Gluckman
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Bellucci, S., Damaj, G., Boval, B. et al. Bone marrow transplantation in severe Glanzmann's thrombasthenia with antiplatelet alloimmunization. Bone Marrow Transplant 25, 327–330 (2000). https://doi.org/10.1038/sj.bmt.1702139

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  • DOI: https://doi.org/10.1038/sj.bmt.1702139

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