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SB82 Hemostasis

From Schwartz Chp. 4: Hemostasis etc.

Factor IX deficiency is also known as ____________. Christmas disease
Coagulation factor deficiency with plasma factor levels < 1% is considered _____________ disease (severity). Severe
Coagulation factor deficiency with plasma factor levels 1-5 % is considered _____________ disease (severity). Moderately severe
Coagulation factor deficiency with plasma factor levels 5-30 % is considered _____________ disease (severity). Mild
For Factor IX deficiency, the preferred produce is _______________ factor IX because of the risk of _________________ with intermediate Factor IX concentrates. Recombinant thrombosis
Up to __________ percent of hemophiliacs with Factor VIII deficiency develop inhibitors. Twenty
The most effective treatment for Hemophilia A patients with high titers of inhibitors is _______________. Recombinant Factor VIIa
Other treatments for Hemophilia A patients with high titers of inhibitors include the following: Porcine factor VIII, prothrombin complex concentrates, activated prothrombin complex concentrates, and recombinant Factor VIIa.
Useful adjuncts for coagulation in Hemophilia A patients include ____________ and __________. Amicar DDAVP
Excess epsilon-aminocaproic acid (Amicar) can lead to ____________. Thrombosis
The most common congenital bleeding disorder is _________________. Von Willebrand disease
Treatment for VWD includes ____________ and __________. Recombinant factor VIII, DDAVP
Genetically, VWD is _________________. (autosomal dominant / autosomal recessive / X-lined dominant / X-linked recessive) Autosomal Dominant
Type I Von Willebrand deficiency is due to a _______________ defect. Quantitative
Type II Von Willebrand deficiency is due to a _______________ defect. Qualitative
Type III Von Willebrand deficiency is due to a _______________ defect. Total deficiency
Factor XI deficiency is also known as _______________. Hemophilia C
Factor XI deficiency is more common in this population. _______________ Ashkenazi Jewish
Treatment for patients with known Factor XI deficiency who present with bleeding or for surgery are treated with ___________. FFP
Each milliliter of FFP contains _________ (amount in units) of factor XI activity. One unit
Spontaneous bleeding in patients with Factor XI deficiency is __________________ (common / rare) Rare
Prior to surgery, patients with Factor XI deficiency may be pre-treated with ____________ to prevent excessive bleeding. Desmopressin acetate
Genetically, Factor VII deficiency is _____________ (autosomal dominant / autosomal recessive / X-lined dominant / X-linked recessive) Autosomal recessive
Bleeding with Factor VII deficiency is uncommon unless plasma levels are less than ___________. Three percent
The half life of recombinant Factor VIIa is approximately _____________. Two hours
The half life of factor VII in FFP is ___________. Four hours
Patients with factor VII deficiency should be treated with ___________ or _____________. FFP, recombinant Factor VIIa
The most common manifestation of Factor VII deficiency is __________ or ___________. Epistaxis, oral mucosal bleeding
Patients with mild hemophilia have normal formation of this structure. ___________ Platelet plug
Bleeding in patients with inherited deficiencies of Factors II / V / X is treated with ________. FFP
Congenital Factor XIII deficiency is a rare autosomal recessive disease that is usually associated with ____________. Severe bleeding diathesis
Bleeding in Factor XII deficiency is usually delayed because: ________. Clots form normally but are susceptible to fibrinolysis
This finding is characteristic of infants born with congenital Factor XIII deficiency: ______________. Umbilical stump bleeding
Women with inherited Factor XIII deficiency are prone to this health hazard: ____________. Spontaneous abortion
This level of Factor XIII is usually adequate for hemostasis: _______. 1-2%
Patients with absent or dysfunctional platelet glycoprotein IIb/IIIa complex have ___________________. Glanzmann thrombasthenia
Bleeding in patients with Glanzmann thrombasthenia should be treated with ________________. Platelet transfusions
This glycoprotein receptor is necessary for platelet adhesion to the subendthelium: ________. Glycoprotein IB IX V receptor
Deficiency or dysfunction of the glycoprotein IB IX V receptor is known as ____________ disease. Bernard Soulier
The most common intrinsic platelet defect is _____________ disease Storage pool
Storage pool disease in platelets involves the loss of ___________. Dense granules and alpha granules
The platelet functional defect involving deficiency of dense granules may occur with this other finding in Hermansky-Pudlak syndrome: _____________. Partial albinism
An isolated defect of the alpha granules of platelets is known as ______________ syndrome. Gray platelet
Mild bleeding associated with storage pool disease may be treated with ____________. Desmopressin acetate
The four physiologic processes that result in hemostasis are: ______________. Vascular constriction, platelet plug formation, fibrin formation, and fibrinolysis.
This substance is produced locally at the site of endothelial injury via the release od arachidonic acid from platelet membranes, and is a potent smooth muscle contrictor. Thromboxane A2
This potent vasoconstrictor is synthesized by injured endothelium and serotonin released during platelet aggregation: __________ Endothelin
The extent of vasoconstriction varies with the degree of ______________. Vessel injury
Up to ____________ percent of circulating platelets may be sequestered in the spleen. Thirty
The average life span of platelets is _____________. 7 - 10 days
Injury to the intimal layer of the vascular wall exposes _______________, to which the platelets adhere. Subendothelial collagen
The main protein in the subendothelium of the vascular wall that promotes platelet adhesion to the wall is _________________. vWF
After platelet adhesion to the injured vascular wall, platelets initiate a _____________ that recruits other platelets from the circulation to seal the disrupted vessel. Release reaction
The principal mediators in platelet aggregation are _________ and __________. ADP, serotonin
This substance promotes platelet aggregation by serving as a bridge for the glycoprotein IIb/IIIa receptor between activated platelets: _____________. Fibrinogen
This protein secreted by platelet alpha-granules stabilizes fibrinogen binding to the platelet surface and strengthens platelet-to-platelet interactions: ____________. Thrombospondin
Fibrinogen levels usually need to be less than ___________ mg/dL to cause prolongation of PT or PTT. fifty
The disease in which factor V is resistant to cleavage by APC and remains an active procoagulant is known as ______________. Factor V Leiden
In children, ITP is typically acute and short-lived, and often follows a _____________ illness. Viral
In contrast to the presentation of ITP in children, ITP in adults is gradual in onset, chronic and is caused by ____________. No identifiable cause
The pathphysiology of ITP in adults is believed to involve both ________________ and __________________. Impaired platelet production, T-cell mediated platelet destruction
Treatment of adult ITP is __________________, and possibly also administration of ____________ and ___________ Withdrawal of the offending medication, corticosteroids, gamma globulin
This is an immunologic disorder in which antibodies against platelet factor 4 result in thrombocytopenia and intravascular thrombosis: ______________. HIT
The platelet count in initial HIT drops within ____________ (days), but may occur as soon as _____________ (days) if due to re-exposure. 5-7 days, 1-2 days
Criteria for HIT: If platelet count falls below _______________ or if it drops by ________ percent in a patient receiving heparin. 100,000 per microLiter, fifty
True or false: All patients who are truly positive for HIT antibody develop clinical HIT. ________ False
HIT may be diagnosed by ______________, which is sensitive but not specific, or by __________, which is specific but not sensitive. ELISA, serotonin release assay
In thrombotic thrombocytopenic purpura, large ______________ molecules interact with platelets, which leads to activation and pro-coagulation state. vWF
The large molecules causing TTP are the result of inhibition of ______________, which usually cleaves these molecules. ADAMTS13
The most effective treatment for TTP is ___________. Plasmapheresis
ADAMTS-13, the inhibited enzyme in TTP, belongs to the following enzyme category: _______________. Metalloproteinase
This condition often occurs secondary to E coli infection or other Shiga-toxin producing bacteria: ____________. HUS
Hemolytic uremic syndrome often results in _____________. Renal failure
A number of patient develop features of both TTP and HUS. This combination is more common in ___________ diseases and with use of ______________ medications. Autoimmune, immunosuppressive
Treatment of medication-induced TTP-HUS involves ___________________. Discontinuation of the offending drug
An important cause of thrombocytopenia, which is often related to portal HTN, sarcoidosis, lymphoma, or Gaucher's disease, is ___________________. Sequestration
One unit of platelets usually increases the platelet count by ______________ per microLiter. ten thousand
Amicar interferes with fibrinolysis by inhibiting activation of ___________. Plasminogen
Thrombocytosis due to polycythemia vera can be reduced by the administration of ________ or ________. Hydroxyurea, anagrelide
Thrombocytopenia in patients with liver disease is typically due to these three causes: _____________, _______________, _______________ Hypersplenism, reduced production of thrombopoeitin, immune-mediated platelet destruction
Immune-mediated thrombocytopenia in cirrhotic patients is usually due to _____________ or __________. HCV, PBC
One major risk associated with repeated platelet transfusions in cirrhotic patient is development of ____________. Antiplatelet antibodies
The absorption of vitamin K is dependent upon __________ production, which is often diminished in cirrhotic patients. Bile
If the fibrinogen level is < 100, administration of _______________ is often helpful to correct coagulopathy. Cryoprecipitate
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