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SB82 Hemostasis
From Schwartz Chp. 4: Hemostasis etc.
Question | Answer |
---|---|
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 |