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27 Cards in this Set
- Front
- Back
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Protein C
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works with Protein S to inactivate Factors V and VIII
activated by Thrombin |
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Protein S
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worked with Protein C as a co-factor to inactivate Factor V and VIII
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Antithrombin III
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serine protease
inactivates IIa mainly also: XIIa, XIa, IXa, Xa lost in NEPHROTIC SYNDROME |
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Nephrotic syndrome
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lose ANTITHROMBIN III in urine
hypercoagulability |
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Factor V Leiden
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pt. mutation in Arg-506
PROTEIN C cannot bind to inactivate 6% Caucasians- 5x heterozygotes and 90x homozygotes for VTE |
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Prothrombin 20210A
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G-->A change at position 20210A of PROTHROMBIN
hypercoagulable results in higher levels of Gactor II |
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hypercoagulable
high levels of Factor II in serum |
Prothrombin 20210A point mutation
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Protein C Deficiency
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vitamin K-dep. inhibitor of coagulation (Factors V and VIII)
heterozygotes = 8-10x risk VTW homozygotes= incompatible with life suceptible to WARFARIN SKIN NECROSIS |
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Warfarin Skin Necrosis
hypercoagulable state |
Protein C deficiency
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Protein S Deficiency
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homozygotes = neonatal purapura fulminans
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AT Deficiency
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rare hypercoagulable state
homozygous not compatible with life heterozygotes= 50x higher risk VTE, esp. w/ pregnancy |
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what catalyzes the activation of ANTITHROMBIN III?
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Heparin and other GAGs
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Paroxysmal Nocturnal Hemaglobinuria
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-mutation inf PIGA and DAF
-both hemolytic (complement dep.-destruction) and hypercoagulable (increase DVT) -worse at night due to resp. acidosis -increased risk of leukemia |
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Tx Hemolytic Uremic Syndrome
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-supportive care
-plasmaphoresis will not work |
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Labs for HUS
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high increase in LDH
decreased platelets nl. PTT nl. Fibrinogen |
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Pathology of HUS
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Siga-like toxin
1. binds to platelets 2. increases release of vWF via endothelial injury |
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TTP
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thrombocytopenia w/ nl. PT and aPTT
def. ADAMTS 13--> cannot cleave vWF multimers clots of platelets and fibrin occlude sm. vessels mechanical shear of RBCs |
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Sx TTP
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-fever
-neurological symptoms -thrombocytopenia -hemorrhage -abdominal pain -renal failure -schistocytes in PBS |
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DIC causes
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-infection
-trauma -surgery -neoplasms |
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HIT Type II
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5-14 days post-heparin
platelet makes PF4 PF4 binds to heparin---> make Ab(heparin complex) -thrombocytopenia -endothelial damage -Fc-dep. platelet aggregation |
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Tx HIT
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discontinue all heparin
Tx: alternatives (hirudin or argatroban) |
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antiphospholipid antibodies diagnosis
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always get 2 confirmatory labs w/ elevated titers
-detection common, need symptoms or high titers |
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Lupus Anticoagulant Differentiation
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-will increase aPTT in vitro
-cardiolipin and B2-M will not change -flood sample w/ phospholipid--> should normalize |
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sex linked hemophilias
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Type A = VIII
Type B= IX |
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TYpe A Hemophilia
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factor VIII absent
sex-linked Tx= cryoprecipate, lyophilized VIII |
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Type B Hemophilia
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factor IX abnormal
sex-linked Tx= lyophilized IX |
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DDAVP
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desmopressin
Tx: vWF def and hemophilia type A (VIII absent) |
