Constitutional thrombocytopathy with subnormal response to thromboxane A2

Br J Haematol. 1981 Jun;48(2):293-303.

Abstract

A new type of congenital platelet dysfunction was found in a young woman presenting a life-long bleeding disorder. The known types of thrombopathia and von Willebrand's disease were excluded by appropriate investigations. The platelets were morphologically normal, underwent normal shape change and contraction and synthesized thromboxane A2 (TXA2) normally. The release reaction was abnormal and the aggregation response to ADP, adrenalin, collagen, thrombin, sodium arachidonate and vasopressin was depressed due to decreased sensitivity of the platelets to prostaglandin endoperoxides and TXA2. Platelet cAMP content was increased.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aspirin / pharmacology
  • Blood Coagulation Tests
  • Blood Platelet Disorders / blood
  • Blood Platelet Disorders / congenital*
  • Blood Platelets / drug effects
  • Blood Platelets / metabolism
  • Female
  • Humans
  • Platelet Aggregation / drug effects
  • Serotonin / metabolism
  • Thromboxane A2 / biosynthesis
  • Thromboxane A2 / pharmacology*
  • Thromboxanes / pharmacology*

Substances

  • Thromboxanes
  • Serotonin
  • Thromboxane A2
  • Aspirin