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Hemostasis
Pathology exam 3
36
Other
Professional
10/09/2007

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Term
What are the three phases of normal hemostasis?
Definition
1.) vascular phase reflex 2.) platelet adhesion to damaged vascular walls and aggregation of platelets to one another 3.) coagulation factor cascade and its fibrinolytic pathways overlaps and intertwines the other phases
Term
When is endothelin released in normal hemostasis and what does it cause?
Definition
it is released directly after injuey and causes vasoconstriction
Term
Normal times for the following are what? 1.) platelet adhesion 2.) platelet aggregation 3.) platelet plug 4.) consolidation 5.) fibrin stabilization
Definition
1.)1-2 seconds 2.) 10 sec 3.) 1-3 minutes 4.) 3-5 minutes 5.) 5-10 minutes
Term
The vascular phase in normal hemostasis consists of four main steps, what are they?
Definition
1.) vasoconstriction of small vessels 2.) flow diversion 3.) contact activation (includes platelets, coagulation factor and complement) 4.) endothelium roles (extrinsic coagulation sequence, counter effect)
Term
T/F platelet phase functionally maintains endothelium, late control of bleeding and stabilization of platelet plug?
Definition
F --> platelet phase does maintain endothelium and helps with stabilization of bleeding but it is initial control of bleeding not late
Term
T/F platelets (wrt adhesion function) stick to exposed ECM and glycosamino-glycans after damage to cells?
Definition
T
Term
T/F Gp2b/3a helps to adhere to vWF
Definition
F Gp1B helps adhere to vWF; Gp2b/3a helps to aggregate platelets by keeping fibrinogen between platelets for connection
Term
T/F A Gp1b deficiency will lead to Glanzmann-thrombasthenia disease?
Definition
F A Gp1b deficiency leads to Bernard-soulier sx and a deficiency in Gp2b/3a leads to Glanzmann-thrombasthenia
Term
What maturation does the common pathway of the coagulation phase allow? (In a general sense)
Definition
Fibrin matures to a thrombus (including platelets)
Term
If the endothelium is damaged and CT is exposed, which pathway is involved, intrinsic or extrinsic?
Definition
intrinsic....extrinsic has to do with tissue thromboplastin initiating
Term
Which system is this describing? fibrin forms sticky threads between platelets that are aggregated, a diffuse gel-like matrix serves as a scaffold for granulation and wound healing, plasminogen helps counter fibrin and tPA is released?
Definition
fibrinolytic system
Term
If there is excessive bleeding after surgery and it is a vascular defect, is it an early or late problem?
Definition
Early; platelet phase and vascular defect are both early problems but a coagulation defect is a late problem
Term
What are the three classification of bleeding disorders?
Definition
1.) nonthrombocytopenic 2.) thrombocytopenic 3.) coagulation disorder
Term
What is the normal bleeding time?
Definition
1-6 minutes
Term
What are the four congenital platelet disorders?
Definition
1.) vWF disease 2.)storage pool disorder 3.) Glanzmans thrombasthenia 4.)Bernard-soulier 5.)ITP
Term
T/F vWF stick to platelets, but not to damaged vessels?
Definition
F vWF stick to both platelets and damaged vessels
Term
Which types of vWF disease are autosomal dominant and which are autosomal recessive?
Definition
type 1&2 are autosomal dominant and type 3 is autosomal recessive
Term
Describe type 1 vWF disease
Definition
decreased amount of vWF, the vWF produced is normal, and all sizes are present
Term
Describe type 2 vWF disease
Definition
abnormal vWF produced, decreased activity
Term
Describe type 3 vWF disease
Definition
no vWF, less han 10% of F-VIII, severe bleeding problems
Term
What are the symptoms and treatment of type 1&2 vWF disease
Definition
usually no severe bleeding, mucosal bleeding may occur, bruising can occur, can bleed excessively at surgery (but can be prevented), treatment with DDAVP (desmopressin)
Term
What are symptoms and treatment of type 3 vWF disease?
Definition
very severe bleeding problems, bleeding into joints, severe bleeding with surgery. Treatment with cryoprecipitates (take blood, spin down and freeze out parts you want): special F-VIII (retains vWF), humate-P, Koate HS
Term
What is ITP?
Definition
Idiopathic thrombocytopenic purpura (inherited platelet disorder); rare disorder that may be acute and half the cases occur with children; children resolve in 70% of cases while adults usually have a chronic course (make sure to look over the treatment of both acute and chronic)
Term
How does an acquired platelet disorder occur?
Definition
dysfunction most commonly caused by meds (interfere with COX: aspirin, NSAIDS, clopidogrel and others)
Term
What are the 2 main screening and lab tests used for platelet disorders?
Definition
Ivy bleeding times and PFA 100(look over details of this test)
Term
What are the congenital coagulation disorders?
Definition
hemophlia A, B, C and vWF
Term
What are the acquired coagulation disorders?
Definition
liver disease, bile obstruction, non-tropical sprue, DIC and caused by meds
Term
What are the different levels of hemophilia A?
Definition
> 25% --> no problem, 5-25% --> mild, 1-5% --> moderate, <1% --> severe
Term
What are the screening tests for hemophilia?
Definition
aPTT --> abnormal and PT,BT & platelelt count are normal
Term
Treatment for hemophilia?
Definition
mild & moderate --> DDAVP and EACA
severe --> DDAVP, EACA and F-VIII replacement
Term
T/F hemophilia B is aka factor XI deficiency and christmas disease?
Definition
F it is known as christmas disease but it is a deficiency in factor IX; while a deficiency in factor XI is hemophilia C
Term
When have an acquired coagulation disorder due to meds an INR measurement is taken. T/F If the INR shows up between 2.0-3.0 the physician needs to reduce the coumadin/heparin dosage ?
Definition
F INR 2-3.0 (little problem with bleeding)
INR 3-3.5 (minor problems)
INR >3.5 (have physician reduce dosage)

A med consult is recommended for all of the above
Term
What is the primary drugs used to prevent deep venous thrombi?
Definition
Low molecular weight heparin
Term
What is fondaparinux?
Definition
a synthetic pentasacharide that mimics the active site of the smallest sequence capable of replicating heparin effects
Term
What are some of the signs to look for in an undiagnosed bleeding disorder?
Definition
petechiae, hemarthrosis, thrombocytopenia, look at tests for bleeding problems: bleeding time (1-6 min), platlet count (140,000-400,000/uL), PTT (25-35 sec; intrinsic pathway), PT (10-14 sec; extrinsic pathway), INR (1.0) These are all normal measurements
Term
Bleeding can be controlled be what means?
Definition
splints, pressure packs, ice, gelfoam with thrombin, oxycel, microfibrillar collagen, tranexamic acid
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