What causes platelet release reaction disorders?

Updated: Aug 05, 2017
  • Author: Perumal Thiagarajan, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

Answer

In disorders of release reaction, platelets fail to secrete proaggregatory ADP following activation. The defects result from either the absence of granules in platelets or the defective storage of ADP. Inherited deficiency of ADP receptor P2Y12, characterized by mild bleeding diathesis, has also been described.

ADP is present in the dense granules of platelets as a storage pool, which is not used in the normal metabolic activity of platelets (in contrast to the metabolic pool). These disorders are often associated with other systemic abnormalities (eg, Hermansky-Pudlak syndrome).

Disorders of secretion and thromboxane synthesis are mild platelet disorders and often respond to desmopressin (DDAVP) infusion, which seems to improve hemostatic function. If severe bleeding is present, these disorders can also be managed effectively with platelet transfusions. Platelet transfusions, however, should be avoided as much as possible because they can induce alloantibodies. Cryoprecipitate has also been reported to be very effective.


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