Abstract
We present the first thrombelastographic descriptions of three patients with δ-storage pool deficiency, a platelet disorder that involves a deficiency of dense granules and moderate bleeding. The patients demonstrated a 49-54% loss of platelet-mediated clot strength over a 1-2-h period after normal thrombus formation. This pattern persisted, with some attenuation of loss of strength following administration of epsilon aminocaproic acid, desmopressin and platelets for tonsillectomy. Assessment of platelet function in patients with platelet granule disorders can be accomplished with thrombelastographic methods in ambulatory and perioperative settings; however, the effects of therapy for this disorder cannot be monitored with thrombelastography without obtaining a blood sample prior to prophylactic hemostatic intervention.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Aminocaproic Acid / administration & dosage
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Aminocaproic Acid / therapeutic use
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Blood Platelets / drug effects*
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Blood Platelets / metabolism
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Cytoplasmic Granules
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Deamino Arginine Vasopressin / administration & dosage
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Deamino Arginine Vasopressin / therapeutic use
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Female
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Fibrinolysis / drug effects
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Hemostasis / drug effects*
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Hemostatics / administration & dosage*
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Hemostatics / therapeutic use
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Humans
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Male
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Platelet Aggregation / drug effects
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Platelet Count
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Platelet Storage Pool Deficiency / blood*
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Platelet Storage Pool Deficiency / diagnosis
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Platelet Storage Pool Deficiency / drug therapy*
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Platelet Storage Pool Deficiency / physiopathology
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Platelet Transfusion
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Thrombelastography / methods*
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Tonsillectomy
Substances
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Hemostatics
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Deamino Arginine Vasopressin
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Aminocaproic Acid