Bone marrow transplantation for Glanzmann's thrombasthenia.

Abstract

Allogeneic matched bone marrow transplantation (BMT) was performed in a patient with type I Glanzmann's thrombasthenia, a rare, inherited bleeding disorder caused by a deficiency in the platelet membrane glycoprotein IIb-IIIa complex. The patient was a 2-year-old girl with a history of frequent hospitalisation. She was successfully transplanted with BM from her HLA-identical sibling. Engraftment was monitored by analysis of the platelet GPIIb-IIIa complex and by RFLP analysis using a minisatellite probe. Complete engraftment was seen at day +25. The patient has been clinically stable for 19 months. It is proposed that BMT is a suitable treatment for this condition where a matched, related donor is available and at an early stage, before the development of anti-platelet antibodies as a result of repeated transfusions.

• Authors: A Johnson, A H Goodall, C J Downie, A Vellodi, D P Michael
• Journal: Bone marrow transplantation (Bone Marrow Transplant) Vol. 14 Issue 1 Pg. 147-50 (Jul 1994) ISSN: 0268-3369 [Print] England
• PMID: 7951102 (Publication Type: Case Reports, Journal Article)

Chemical References

• Platelet Membrane Glycoproteins

Topics

• Blood Platelets (metabolism)
• Bone Marrow Transplantation
• Child, Preschool
• Female
• Heterozygote
• Homozygote
• Humans
• Phenotype
• Platelet Membrane Glycoproteins (genetics, metabolism)
• Polymorphism, Restriction Fragment Length
• Thrombasthenia (blood, genetics, therapy)
• Tissue Donors
• Transplantation, Homologous