BONE-MARROW TRANSPLANTATION FOR GLANZMANNS-THROMBASTHENIA

Allogeneic matched bone marrow transplantation (BMT) was performed in a patient with type I Glanzmann's thrombasthenia, a rare, inherited bl eeding disorder caused by a deficiency in the platelet membrane glycop rotein IIb-IIIa complex. The patient was a 2-year-old girl with a hist ory of frequent hospitalisation. She was successfully transplanted wit h BM from her HLA-identical sibling. Engraftment was monitored by anal ysis of the platelet GPIIb-IIIa complex and by RFLP analysis using a m inisatellite probe. Complete engraftment was seen at day +25. The pati ent has been clinically stable for 19 months. It is proposed that BMT is a suitable treatment for this condition where a matched, related do nor is available and at an early stage, before the development of anti -platelet antibodies as a result of repeated transfusions.