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storage pool disease
Pathology:
- congenital platelet defects
- decrease or absence of dense granules & thus a marked decrease in platelet ADP & a lesser decrease in ATP.
Clinical manifestations:
- mild to moderate rather than severe bleeding disorder
Laboratory:
1) variable platelet aggregation abnormalities
a) decrease 2nd wave aggregation in response to epinephrine, a low concentration of ADP & collagen
b) normal aggregation with a high concentration of ADP (in contrast to Glanzmann's thrombasthenia)
2) prolonged bleeding time
Management:
- platelet transfusions for active bleeding as needed
- epsilon-aminocaproic acid for active bleeding as needed
- some patients respond to desmopressin [2]
Related
bleeding time
General
platelet disorder; thromboasthenia
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 439
- Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18.
American College of Physicians, Philadelphia 1998, 2015, 2018.