PLATELET DISORDERS Flashcards
disorder of platelet adhesion - deficiency of glycoprotein Ib/Ix
bernard-soulier syndrome
disorders of platelet aggregation - deficiency of glycoprotein IIb/IIIa
Glanzmann-Thrombasthenia
alpha or dense granules deficiency
disorders of platelet secretion
platelets fail to promote activation of the blood clotting proteins
disorders of platelet procoagulant activity
drugs like aspirin, non-steroidal anti-inflammatory drugs like indomethacin, ibuprofen
acquired platelet function disorders
Defect in adherence - The glycoprotein (Ib) receptor for VIII:Vwf is absent on platelet membrane and the Rx: platelets transfusion
Bernard-Soulier Syndrome
Defect in aggregation - the platelets lack surface glycoprotein receptor (IIb, IIIa) necessary for binding fibrinogen (fibrinogen receptor) and bleeding is prolonged
Glanzmann Thrombasthenia
congenital abnormalities of platelets can be divided into ________ and those of _______
disorders of platelet production ; platelet function
congenital abnormalities all are very ____
rare
congenital abnormal in general, cause moderate to severe _______
severe problems
autosomal recessive preleukemic condition which often presents as thrombocytopenia with skeletal or genitourinary abnormalities
Fanconi’s Anemia
congenital abnormalities laboratory features
- abnormal chromosomal fragility
- the condition can be cured with bone marrow transplantation (BMT)
thrombocytopenia clinical symptoms typically not seen until ______
<100 x 10^9/L
thrombocytopenia more often _____
<50 x 10^9/L
thrombocytopenia spontaneous bleeding occurs ____
<20 x 10^9 / L
thrombocytopenia life-threatening _______
<10 x 10^9/L
thrombocytopenia clinical manifestation
- petechiae
- menorrhagia
- spontaneous bruising
- fatal bleeding into the CNS may occur
- spontaneous bleeding in GI-tract, GU-tract and nose
- prolonged BT but normal coagulation tests
thrombocytopenia is due to
- deficient platelet production
- abnormal platelet distribution (splenic sequestration)
- increased destruction
- secondary to an underlying disease
- most common cause of excess or abnormal bleeding
thrombocytopenia
thrombocytopenia platelet count
< 100,000 cu mm
platelet count <50, 000
variable risk