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- Title
Bone marrow transplantation in severe Glanzmann’s thrombasthenia with antiplatelet alloimmunization.
- Authors
Bellucci, S; Damaj, G; Boval, B; Rocha, V; Devergie, A; Agha, I Yacoub; Garderet, L; Ribaud, P; Traineau, R; Socié, G; Gluckman, E
- 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.
- Subjects
BONE marrow transplantation; HEMORRHAGE
- Publication
Bone Marrow Transplantation, 2000, Vol 25, Issue 3, p327
- ISSN
0268-3369
- Publication type
Article
- DOI
10.1038/sj.bmt.1702139