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platelet disorder; thromboasthenia

Etiology: 1) platelet adhesion defects a) von Willebrand's disease b) Bernard Soulier disease 2) platelet aggregation defects - Glanzmann's thrombasthenia 3) platelet release defects a) anti-platelet drugs 1] non-steroidal anti-inflammatory drugs (NSAIDs) (most common acquired qualititative platelet defect) 2] glycoprotein IIb/IIIa inhibitors - abciximab, eptifibatide, tirofiban 3] P2Y12 receptor inhibitors - clopidogrel, ticagrelor, prasugrel b) granule storage pool defects 1] congenital 2] acquired c) uremia d) platelet coating 1] penicillin 2] paraproteins 4) platelet coagulant defect - Scott's syndrome 5) other congenital platelet defects [1] - Wiskott-Aldrich syndrome - gray platelet syndrome - congenital cyclo-oxygenase inhibition/deficiency 6) other acquired platelet defects - liver disease - myeloproliferative disease - post cardiac bypass 7) disorders associated with thrombocytosis a) essential thrombocythemia b) polycythemia rubra vera 8) thrombocytopenia a) thrombotic thrombocytopenic purpura - hemolytic uremic syndrome b) heparin-induced thrombocytopenia Epidemiology: 1) only von-Willebrand's disease is common 2) all other platelet disorders (excepting drug induced) are rare Clinical manifestations: - mucosal bleeding is characteristic Laboratory: 1) complete blood count (CBC) - platelet count may be normal 2) bleeding time may be prolonged 3) INR & aPTT normal 4) von Willebrand factor (vWF) may be abnormal 5) platelet aggregometry may be abnormal 6) see ARUP consult [3] Management: - epsilon-aminocaproic acid used to treat platelet defects due to - Bernard Soulier disease - Glanzmann's thrombasthenia - Wiskott-Aldrich syndrome - gray platelet syndrome - granule storage pool defects - uremia - liver disease

Related

platelet platelet count

Specific

acquired platelet aggregation disorder Bernard-Soulier disease (giant platelet syndrome, benign Mediterranean macrothrombocytopenia) Epstein syndrome Glanzmann's thrombasthenia gray platelet syndrome macrothrombocytopenia with sensorineural deafness Quebec platelet disorder Sebastian syndrome storage pool disease thrombocytopenia thrombocytosis (thrombocythemia) von-Willebrand's disease (vWD)

General

hematologic disease (blood disorder, blood dyscrasia)

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018
  2. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 321
  3. ARUP Consult: Functional Platelet Disorders The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/functional-platelet-disorders
  4. Burrows RF. Platelet disorders in pregnancy. Curr Opin Obstet Gynecol. 2001 Apr;13(2):115-9. PMID: 11315863
  5. Konkle BA. Acquired disorders of platelet function. Hematology Am Soc Hematol Educ Program. 2011;2011:391-6. Review. PMID: 22160063
  6. Mohan G, Malayala SV, Mehta P, et al. A comprehensive review of congenital platelet disorders, thrombocytopenias and thrombocytopathies. Cureus. 2020;12:e11275. PMID: 33274150