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23 Cards in this Set
- Front
- Back
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ACUTE ITP -describe
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sudden onset
usually affects children often post viral usually self-limiting w/i 6 months treated with steroids |
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CHRONIC ITP -describe
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autoantibodies against IIb-IIIa or Ib usually IgG surface Abs opsonize platelets and render them susceptible to phagocytosis
female: male = 3:1 insidious onset petechiae and ecchymoses Tx: corticosteroids relapse may lead to splenectomy Lab findings: decreased platelet count, normal/increased megs in BM, large platelets on PBS, bleeding tim is prolonged, PT and PTT are prolonged |
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heparin induced thrombocytopenia
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due to antibody that recognize heparin bound to PF4
1st time plt decrease on average 10 days after thrombocytopenia in 1-5% in future use non-heparin anticoagulant can also develop thrombosis |
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HIV associated thrombocytopenia
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impaired production and increased destruction
CD4 on platelets means they can be infected w/ HIV as well as T cells infected cell prone to apoptosis autoantibodies to gp IIb-IIIa may be immune complexes Tx w/ AZT or interferon |
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thrombotic thrombocytopenic purpura TTP
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acquired: drugs, pregnancy, SLE, infections, malignancy
inherited lack of vWF cleaving enzyme results in accumulation of ULvWF--> microthrombi, endothelial cell injuy |
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Hemolytic uremic syndrome
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normal levels of ADAMTS 13
metalloprotease in endothelial cells E.coli 0157:H7 initiates platelet activation and aggregation |
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Bernard-Soulier syndrome
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AR, GpIb deficiency
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Glanzmann thrombasthenia
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ER dont aggregate in presence of ADP, collagen, epi, thrombin
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storage pool disorders
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grey platelet syndrome
delta storage pool deficiency Chediak-Higashi WIskott-Aldrich |
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acquired functional defects
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drug induced: aspirin
uremia liver disease paraneoplastic platelet dysfunction |
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disorders related to abnormal clotting factors
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bleeding, different than in platelet disorders
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what is the only factor that doesnt cause bleeding?
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12
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Protein C and Protein S inactivate
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factor VIIIa
factor VIII normally circulates as a complex w/ vWF |
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Hemophilia A
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quantitative deficiency of factor VIII
most common hereditary diseases assoc with serious bleeding varying degrees of severity based on: <1% severe disease 2-5% moderate disease 6-50% mild disease X linked recessive |
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factor 8 inhibitors
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about 15% develop antibodies
more severe, higher risk most are IgG 4 |
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treatment for factor 8 inhibitors
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prothombin complexes
activated prothombin compelxes recombinant factor 8 immunonosuppressive agents IV gamma globulin |
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factor XI deficiency
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part of intrinsic pathway
once activated it along w/ FVIII catalyzes activation Factor9 to 9a AR increased frequency in Ashkenazi Jews may be asymptomatic even if deficiency is severe |
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vWD
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most common inherited bleeding disorder
facilitates platelet adhesion to vessel wall plasma carrier and stabilizer 1 and 3 do not have wVF, 2 has defective vWF |
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lab findings
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normal platelet
prolonged bleeding Ristocetin cofactor activity reduced ristocetin induced platelet aggregation antibiotic Ristocetin cause von Willeband factor to bind platelet receptor glycoprotein Ib so when it is added to normal blood to causes agglutination in von willebrand disease, where von willebrand factor is absent or defective/abnormal agglutination occurs |
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dense bodies contain
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ADP
ATP serotonin calcium |
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alpha granules
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beta thromboglobulin
PF4 PDGF thrombospondin coagulation factors |
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Henoch-Schonlein purpura
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similar to IgA nephropathy
usually children invove skin, connective tissues, GI joints kidneys |
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connective tissue disorder
AD hyperelasticity of skin, poor wound healing vascular lesions: aortic dissection, aneurysm, vessel fragility |
Ehlers Danlos syndrome
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