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

• variable platelet aggregation abnormalities
  • decrease 2nd wave aggregation in response to epinephrine, a low concentration of ADP & collagen
  • normal aggregation with a high concentration of ADP (in contrast to Glanzmann's thrombasthenia)
• prolonged bleeding time

Management

• platelet transfusions for active bleeding as needed
• epsilon-aminocaproic acid for active bleeding as needed
• some patients respond to desmopressin

More general terms

• platelet disorder; thromboasthenia

Additional terms

• bleeding time