Familial and constitutional bleeding disorder due to platelet cyclo-oxygenase deficiency

American Journal of Hematology
M H HorellouM Samama

Abstract

Three family members from two successive generations had a bleeding tendency. Their template bleeding time was prolonged and platelet aggregation induced by ADP and adrenaline showed no second wave; collagen at low to moderate concentrations failed to aggregate and release ATP, whereas higher amounts aggregated and released. Aggregation and release due to thrombin, ristocetin, and synthetic epoxy derivatives (U 44069 and U 46619) were normal. Arachidonate (AA) was inactive, and was not converted either in thromboxane (TX) A2 activity evaluated on the rabbit aorta strip, nor in TXB2 evaluated by radioimmunoassay and by radiochromatography. The parallel impairment of TXB2 and PGE2 formation by the patient's platelets are compatible with a platelet cyclo-oxygenase deficiency. This study suggests that transmission is autosomal dominant, and confirms that cyclo-oxygenase is not needed for aggregation and ATP release by high amounts of collagen.

References

Sep 1, 1977·Proceedings of the National Academy of Sciences of the United States of America·R R GormanF A Fitzpatrick
Apr 1, 1975·Proceedings of the National Academy of Sciences of the United States of America·C MalmstenB Samuelsson
Feb 1, 1964·The Biochemical Journal·V P SkipskiM Barclay
Feb 1, 1972·British Journal of Haematology·J F MustardM A Packham
Apr 26, 1980·Lancet·F I ParetiG Galli
Jun 1, 1981·The Journal of Clinical Investigation·K K WuY C Chen
Jun 1, 1981·British Journal of Haematology·M SamamaM Dechavanne

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Citations

Jul 1, 1989·Baillière's Clinical Haematology·R M Hardisty
Feb 1, 2014·Journal of Oral and Maxillofacial Surgery : Official Journal of the American Association of Oral and Maxillofacial Surgeons·Jesse C Doscher, Fedele N Volpe
Nov 17, 2006·British Journal of Haematology·Paula H B Bolton-MaggsUNKNOWN UKHCDO
Apr 1, 1991·Baillière's Clinical Haematology·V S BlanchetteC Turner
Sep 27, 2005·Thrombosis Research·Yesim DargaudClaude Negrier
Oct 4, 2003·Nature Reviews. Drug Discovery·Shaun P Jackson, Simone M Schoenwaelder
Apr 15, 1990·Cancer·T J PanellaC S Greenberg
Jul 23, 2003·Journal of Thrombosis and Haemostasis : JTH·A K Rao
Jan 1, 1993·Platelets·J V Lloyd
Jun 26, 2020·Platelets·Verónica Palma-BarquerosJosé Rivera
Jan 1, 1996·Critical Reviews in Oncology/hematology·I Fuse

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