Hereditary Thrombocytopenia with Excessively Prolonged Bleeding Time
- 1 August 1969
- journal article
- Published by Wiley in Scandinavian Journal of Haematology
- Vol. 6 (4) , 250-261
- https://doi.org/10.1111/j.1600-0609.1969.tb02405.x
Abstract
A report is given of a hereditary bleeding disorder characterized by thrombocytopenia and excessively prolonged bleeding time.The disorder was inherited as an autosomal dominant trait, and segregated independently of the ABO, Rh, MNSs and Duffy blood group systems, and probably of the ABH secretor genes.The patients' own platelets had a shortened survival in the patients' own circulation, indicating an increased destruction. The increased destruction was not due to an intrinsic defect of the patients' platelets, because the platelets survived normally when transfused to a normal recipient. The patients must therefore have some factor or mechanism in their blood or vascular system which shortens the life‐span of their own platelets as well as that of transfused normal platelets.Another striking finding was the unproportionate increase of the bleeding time. The moderately reduced platelet count and normal AHG exclude von Willebrand's disease. The presently reported bleeding disorder did not show the characteristics of the thrombasthenic or the thrombopathic group of disorders.The presently reported haemorrhagic disorder does not fit into any of the previously described ones.Keywords
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