Term
| steps of primary hemostasis |
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Definition
1- vasoconstriction
2- platelet adhesion
3- platelet degranulation
4- platelet aggregation |
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Term
| vasoconstriction is mediated by _________ and _________ |
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Definition
| neural stimulation and endothelia release |
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Term
| vWF binds to _________ receptor on platelets |
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Definition
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Term
| vWF is derived from _________ and _________ |
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Definition
| Weibel-Palade bodies of endothelial cells and alpha-granules of platelets |
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|
Term
| platelets release _________ and _________ |
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Definition
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Term
|
Definition
| exposure of GPIIb/IIIa receptor on platelets |
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Term
| Platelets aggregate via _________ |
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Definition
| GPIIb/IIIa using fibrinogen |
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|
Term
| clinical features of disorders of primary hemostasis |
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Definition
| mucosal bleeding and skin bleeding |
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Term
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Definition
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Term
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Definition
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Term
| most common cause of thrombocytopenia in children and adults |
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Definition
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Term
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Definition
| produces auto-antibodies via plasma cells and consumes antibody-bound platelets via macrophages |
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Term
|
Definition
| weeks after viral infection or immunization; resolves |
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Term
|
Definition
| women of childbreaing age; may be in offspring |
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Term
|
Definition
| decreased platelet count; normal PT/PTT; increased megakaryocytic |
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Term
|
Definition
| corticosteroids; IVIG; splenectomy |
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|
Term
| TTP is due to decreased _________ |
|
Definition
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|
Term
|
Definition
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|
Term
| clinical features of HUS and TTP |
|
Definition
| skin and mucosal bleeding, microangiopathic hemolytic anemia, fever, renal insufficiency, CNS abnormalities |
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|
Term
| lab findings for TTP and HUS |
|
Definition
| increased bleeding time, normal PT/PTT, anemia with schistiocytes, increased megakaryocytic |
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Term
|
Definition
| plasmapheresis and corticosteroids |
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Term
| Deficiency in Bernard-Soulier is a deficiency in _________ |
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Definition
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|
Term
|
Definition
| GPIIb/IIIA; platelet aggregation |
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|
Term
| How ASA inhibits platelets |
|
Definition
| irreversibly inhibits cyclooxygenase; lack of TXA2 impairing aggregation |
|
|
Term
| how Uremia inhibits platelets |
|
Definition
| both adhesion and aggregation |
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|
Term
| activation of coagulation cascade requires _________, _________ and _________ |
|
Definition
| exposure to an activating substance, phospholipid surface of platelets, calcium |
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|
Term
| _________ activates the extrinsic pathway |
|
Definition
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|
Term
| _________ activates intrinsic pathway |
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Definition
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|
Term
| clinical features of secondary hemostasis |
|
Definition
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|
Term
| lab findings in disorders in secondary hemostasis |
|
Definition
PT = extrinsic
PTT = intrinsic |
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|
Term
| factor _________ is deficient in hemophilia A |
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Definition
|
|
Term
| lab findings in hemophilia A |
|
Definition
| increased PTT, normal PT; decreased FVIII; normal platelet count and bleeding time |
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|
Term
|
Definition
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|
Term
| factor _________ is deficient in hemophilia B |
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Definition
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|
Term
| most comman coagulation factor inhibitor |
|
Definition
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|
Term
| lab finding characteristic of coagulation factor inhibitors |
|
Definition
| PTT does NOT correct with mixing with normal plasma and thep patient's plasma |
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|
Term
| most comon inherited coagulation disorder |
|
Definition
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|
Term
| lab findings in vWF disease |
|
Definition
| increased bleeding time, increased PTT, abnormal ristocetin test |
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|
Term
| why is PTT increased in vWF disease |
|
Definition
| vWF normally stabilizes FVIII so decreases FVIII half life; no clinical findings though |
|
|
Term
| why is there an abnormal ristocetin test in vWF disease |
|
Definition
| ristocetin induces latelet aggregation by causing vWF to bind GPIb; lack of vWF --> impaired aggregation |
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|
Term
|
Definition
| demopressin= increases release of vWF from Weibel-Palade bodies |
|
|
Term
| vitamin K is activate by _________ |
|
Definition
|
|
Term
| vitamin K carboxlates: _________, _________, _________, _________, _________, _________ |
|
Definition
| factors II, VII, IX, X, protein C and protein S |
|
|
Term
| vitamin K deficiency occurs in _________, _________ and _________ |
|
Definition
| newborns, long-term antibiotic use and malabsorption |
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|
Term
| two other causes of abnormal secondary hemostasis |
|
Definition
| liver failure and large-volume transfusion |
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|
Term
| describe heparin-induced theombodytopenia |
|
Definition
| heparin causes platelet destruction and then the destroyed platelets activate the remaining platelets, causing thrombosis |
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|
Term
|
Definition
obstetric (tissue thromboplastin in amniotic fluid)
sepsis (cytokines induce endothelial cells to make tissue factor)
adenocarcinoma (mucin)
APL (primary granule release)
rattlesnake bite (venom) |
|
|
Term
|
Definition
| decreased platelet count, increased PT/PTT, decreased fibrinogen, microangiopathic hemolytic anemia, elevated D-dimer |
|
|
Term
|
Definition
| address underlying cause, transfuse blood products, and cryoprecipitate |
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|
Term
| describe normal fibrinolysis |
|
Definition
| tPA converts plasminogen to plasmin; plasmin cleaves fibrin and serum fibrinogen, destroys coag factors and blocks platelet aggregation; alpha-2-antiplasmin inactivate s plasmin |
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|
Term
| examples of fibrionlysis disorders |
|
Definition
| radical prostatectomy and cirrhosis of liver |
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|
Term
| lab findings in disorders of fibrinolysis |
|
Definition
| increased PT/PTT, increased bleeding time, normal platelet count, increased fibrinogen split products but NOT D-dimer |
|
|
Term
| histologic characteristics of thrombus |
|
Definition
| lines of Zahn, and attachment to vessel wall |
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|
Term
|
Definition
| disruption of blood flow, endothelial cell damage, and hyper coagulable state |
|
|
Term
| examples of disruption in normal blood flow |
|
Definition
| immobilizaiton, cardiac wall dysfunction, aneurysm |
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|
Term
| how endothelial cells prevent thrombosis |
|
Definition
| block exposure, PGI2 and nO, heparin-like molecules, tPA, thrombomodulin |
|
|
Term
|
Definition
| vasodilation and inhibition of aggregation |
|
|
Term
|
Definition
| augments AtiiI which inactivates thrombin and coag factors |
|
|
Term
|
Definition
| redirect thrombin to activate protein C --> inactivates factors V and VIII |
|
|
Term
| cases of endothelial damage |
|
Definition
| atherosclerosis, vasculitis and high levels of homocystein |
|
|
Term
| causes of increased homocystein |
|
Definition
| Vitamin B12 and folate deficiency and cystathionine beta synthase deficiency |
|
|
Term
|
Definition
| thrombosis, mental retardation, lens dislocation and long slender fingers |
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|
Term
| presentation of hyper coagulable state |
|
Definition
| recurren DVTs and DVT at young age |
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|
Term
| causes of hyper coagulable state |
|
Definition
| protein C and S deficiency, factor V leiden, prothrombin 20210A, ATIII deficiency, oral contraceptives |
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|
Term
| why increased risk for warfarin skin necrosis in protein C or S deficiency |
|
Definition
| initial warfarin therapy results in temporary deficiency of proteins C, S and factors II, VII IX and X; in preexisting C or S deficiency, a severe deficiency is seen at the onset of warfarin , increasing risk for thrombosis |
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|
Term
| most common inherited cause of hyercoagulable state |
|
Definition
|
|
Term
| PTT doesn't rise in ATIII deficiency with what drug? |
|
Definition
|
|
Term
| fat embolus clinical features |
|
Definition
| dyspnea, petichiae on skin of chest |
|
|
Term
|
Definition
|
|
Term
|
Definition
| decompression sickness; nitrogen gas precipitates out; or laparoscopic surgery |
|
|
Term
| gas embolus clinical presentation |
|
Definition
| joint and muscle pain ('bends') and respiratory symptoms "chokes" |
|
|
Term
| amniotic fluid embolus presentation |
|
Definition
| SOA, neurologic syptoms and DIC |
|
|
Term
| amniotic fluid embolus histologic features |
|
Definition
| squamous cells and keratin debris |
|
|
Term
|
Definition
lung has dual blood supply
embolus is usually small |
|
|
Term
| presentation of PE of large/medium vessel with pre-existing cardiopulmonary compromise |
|
Definition
| SOA, hemoptysis, pleuritic chest pain, leural effusion, V/Q mismatch, fascular filling defect on spirl CT, dopper to detect CVT, D-dimer elevated, hemorrhagic, wedge-shaped infarct |
|
|
Term
| where systemic embolus arises from |
|
Definition
|
|
Term
| most common site of systemic embolism |
|
Definition
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