Journal
HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA
Volume 27, Issue 3, Pages 585-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.hoc.2013.02.005
Keywords
Inherited platelet function disorders; Platelet secretion disorders; Storage pool deficiency; Signal transduction defects; Scott syndrome
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Funding
- [NIH-RO1HL109568]
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Inherited disorders of platelet function are characterized by highly variable mucocutaneous bleeding manifestations. The platelet dysfunction arises by diverse mechanisms, including abnormalities in platelet membrane glycoproteins, granules and their contents, platelet signaling and secretion mechanisms: thromboxane production pathways and in platelet procoagulant activities. Platelet aggregation and secretion studies using platelet-rich plasma currently form the primary basis for the diagnosis of an inherited platelet dysfunction. In most such patients, the molecular and genetic mechanisms are unknown. Management of these patients needs to be individualized; therapeutic options include platelet transfusions, 1-desamino-8D-arginine vasopressin (DDAVP), recombinant factor Vila, and antifibrinolytic agents.
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