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

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.

• Authors: S Bellucci, G Damaj, B Boval, V Rocha, A Devergie, I Yacoub-Agha, L Garderet, P Ribaud, R Traineau, G Socié, E Gluckman
• Journal: Bone marrow transplantation (Bone Marrow Transplant) Vol. 25 Issue 3 Pg. 327-30 (Feb 2000) ISSN: 0268-3369 [Print] England
• PMID: 10673707 (Publication Type: Case Reports, Journal Article)

Chemical References

• HLA Antigens
• Isoantibodies
• Platelet Glycoprotein GPIIb-IIIa Complex

Topics

• Adolescent
• Blood Platelets (immunology)
• Bone Marrow Transplantation
• Child
• Female
• HLA Antigens (immunology)
• Hemorrhage
• Humans
• Isoantibodies (blood)
• Male
• Nuclear Family
• Pedigree
• Platelet Glycoprotein GPIIb-IIIa Complex (immunology)
• Thrombasthenia (blood, immunology, therapy)