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heme 201-3
68k heme 201
52
Medical
Not Applicable
02/02/2012

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Cards

Term
thrombocytopenia
Definition

abnormally low plt ct

most common cause of abnormal bleeding

plt ct < 50,000 then significant bleeding occurs

spontaneous bleeding into skin, mucous membranes, and brain causes intracranial hemorrhage when plts < 10,000

Term
thrombocytosis
Definition
high plt ct
Term
chronic idiopathic thrombocytopenia
Definition

found in adults

affects more women than men

history of petechiae, epistaxis, abnormal bleeding, recent viral infection

plt ct: 30,000- 80,000

duration: months- yrs

therapy: corticosteriods or splenectomy

Term
acute ITP
Definition

found in children

male/female incidence the same

abrupt onset

50% occur after viral infection, usually in winter

plt count <20,000

duration: 2-6 weeks

Term
thrombocytosis
Definition

abnormally high plt ct: >450,000

can cause strokes due to plt clots

Term
primary throbocytosis
Definition

marked and persistent elevation in plt ct and impaired plt function

seen in chronic myeloproliferative disorders

essential thrombocythemia has highest plt ct: values can exceed 1 million

Term
secondary (reactive) thrombocytosis
Definition

platelet function is normal, although increased plt levels may last for days to weeks

chronic inflammatory diseases such as rheumatoid arthritis; splenectomy; rebound thrombocytosis in recovering alcoholics or patients on chemotherapy

iron deficiency anemia

Term
von williebrand's disease
Definition
decrease or abnormally in von Williebrand factor (VIII) resulting in defect in plt adhesion
Term
von williebrand's factor lab findings
Definition

prolonged bleeding time

plt count is normal to slightly elevated

aptt may be abnormal

pt is normal

Term
causes of coagulations factor disorders
Definition

defect in the synthesis of the factor

-leads to a decreased concentration

synthesis of a defective factor

- leads to abnormal amounts of an inactive or abnormally functioning factor

Term
fresh frozen plasma (ffp)
Definition

contains all coagulation factors normally found in the circulating blood

treatment for coagulations factors disorders

Term
cyroprecipitate
Definition

contains vwf, fibrinogen, factorsVII and XIII

factor VIII concentrate and ATIII concentrate

treatment for coagulation factor disorders

Term
factor I deficiency
Definition

hereditary deficiencies:

hypofibrinogenemia

dysfibrinogenemia

impaired production

excess utilization

fribinogen destruction

complication of surger

Term
hypofibrinogenemia
Definition

plasma fibrinogen less than 100mg/dL

no major bleeding

prolonged PT, APTT, and Thrombin time

 treat with cyroprecipitate and FFP

Term
dysfibrinogenemia (hereditary)
Definition

produces structurally abnormal fibrinogen

show few syptoms other than mild hemorrhage

PT and APTT are slightly prolonged and thrombin time is prolonged

quantitative fribrinogen is usually normal but functional fibrinogen is prolonged

 

treated with cryoprecipitate and FFP

Term
Hemophilia
Definition

group of disorders in which a particular clotting factor is decreased

two types: hemophilia A, factor VIII deficiency

hemophilia B, factor IX deficiency

both are sex-linked recessive disorders:

mother carries the abnormal gene and passes the gene to her son

Term
hemophilia A (FACTOR VIII deficiency)
Definition

deficiency or dysfunctional of procoagulant portion (VIII:C) of the factor VIII molecule

inherited as a sex-linked recessive trait

 

Term
hemophilia A lab findings
Definition

clotting factors:

severe hemophilia- less than 1% factor VIII:C activity: moderate hemophilia (2-5%): if factor VIII:C doesnt fall below 20%, spontaneous bleeding usually does not occur

normal plt ct, bleeding time, pt

prolonged APTT

Term
hemophilia b
Definition

also known as christmas disease

congenital deficiency

acquired deficiency: coumadin drugs, vitamin k deficiency, liver disease

Term
hemophilia b lab results
Definition

normal plt ct, bleeding time, and pt

prolonged APTT

 

Treatment: ffp, factor XI complex, factor XI concentrates

Term
factor V Deficiency
Definition

congenital deficiency: patients show varying degrees of bleeding and bruising

prolonged pt and aptt

acquired deficiencies associated with DIC, liver disease and acute leukemia

treated ffp

Term
factor x deficiency
Definition

acquired deficiency: liver disease, vitamin k deficiency, coumadin

rare

lab results: prolonged pt and aptt

Term
factor XI D  deficiency
Definition

congenital deficiency

bleeding less severe than hemophilia a and spontaneous hemorrhaging is rare

lab results: normal pt, prolonged aptt

Term
factor XIII deficiency
Definition
hemorrhage following surgery or trauma, intravascular bleeding, easy bruising and possible bleeding into the central nervous system
Term
lupus inhibitor
Definition

most frequently occuring inhibitor

interferes with the phospholipids portion of the complex which converts prothrombin to thrombin

 

Term
complications of lupus inhibitor
Definition

bleeding is uncommon, thromobosis in 30% of patients

pregnant women with lupus anticoagulants run a higher risk of intrauterine deaths or spontaneous abortions

Term
factor VIII inhibitor
Definition

treatment of these patients is difficult because replacement therapy with factor VIII may produce an increased titer of inhibitor

this neutralizes or inactivates the factor VIII given

Term
liver disease
Definition

decreased level of vitamin K dependant factors

decreased factor v and the contact factors

abnormal fibrinolysis

thrombocytopenia and qualitative platelet disorders

Term
liver disease lab findings
Definition

variable lab findings

abnormal pt aptt bleeding time and plt ct

fibrinogen: varies

prolonged thrombin time due to functionally abnormal fibrinogen

 

treatment: administer vitamin k or antifibrinolytic agents

transfusion of FFP/ plts

Term
disseminated intravascular coagulation
Definition

a pathological form of coagulation that is diffused rather than localized

characterized by generalized bleeding and shock

Term
dic lab results
Definition

increased pt and aptt

decreased fibrinogen and plt ct

positvie fdp and d-dimer

fragmented rbcs in peripheral

 

treatment: eliminate underlying disorder

Term
giant platelets
Definition

large platelets greateer in size than a normal rbc

associated with disease states having increased rate of platelet production

Term
thrombin time test
Definition

measures the availability of functional fibrinogen (factor I)

Principle: thrombin added to plasma ; length of time for fibrin clot to form is the thrombin time

refererence range: 15.0-21.0 seconds

Term
causes of prolonged thrombin time
Definition

fibrinogen level below 100 mg/dL

impaired fibrinogen function

fibrin degradation products

thrombolytic agents used to monitor streptokinase and urokinase therapy

Term
fibrinogen test
Definition

purpose: measures concentration of fibrinogen (factor I)

reference  range: 220- 498 mg/dL

Term
fibrinogen test principle
Definition

excess thrombin is added to a specimen of diluted plasma and the clotting time is noted

concentration of fibrinogen in the unknown sample is determined by comparing results with clotting times of standard reference plasma dilutions containing known amounts of fibrinogen

clotting time of the plasma is inversely proportional to the concentration of fibrinogen in the specimen

Term
causes of increased and decreased plasma fibrinogen
Definition

increased: pregnancy, acute inflammation and infections,systemic fibrinolysis, severe liver disease, 

 decreased: dic,congenital afibrinogenemia, hypofibrinogenemia

Term
fdp test
Definition

purpose: detects the presence of fibrinogen degradation products which are markers of fibrinolytic activity

reference range: <10ug/ml (adult)

Term
principle of fdp test
Definition

whole blood is added to thrombin( ensures complete clotting)and soy bean inhibitors ( prevents breakdown of fibrin)

after complete clotting, the patients serum is diluted and mixed with latex particles coated with anti-fdp. The mixture is rocked on a slide for 2 minutes.

IF FDP IS PRESENT LATEX PARTICLES WILL AGGLUTINATE

Term
clinical correlations of fdp test
Definition

primary fibrinolysis

dic with secondary fibrinolysis

thrombosis states

alcoholic cirrhosis of the liver

late pregnancy

Term
d-dimer test
Definition

purpose: detects the presence of fdps containg cross-linked d-dimer

indicates that a stable fibrin clot has lysed

reference range: 2-6ng/dL

Term
principle of d-dimer test
Definition

a dilution of the patients serum is mixed with latex particles coated with monoclonal antibodies to the d-dimer

if fdp containg the d-dimer portion is present in the patient's plasma the latex particles will agglutinate

Term
clinical correlations of increased d-dimers
Definition

pulmonary embolism, deep vein thrombosis, myocardial infarction, ischemic strokes, and arterial thromboembolism

dic with secondary fibrinolysis

sickle cell disease

Term
bleeding time test
Definition

purpose: screening test for plt disorders and von williebrand's disease

affected by: plt ct and ability of plts to form a plug

reference range: 2- 9 min

Term
clinical correlations of prolonged bleeding time
Definition

abnormalities: plt ct <50,000/uL; dysfunctional plts; von williebrand's disease

ingestion of aspirin, anticoagulants, other drugs

technical errors: puncture of larger blood vessel, blood pressure cuff to tight or loose, disturbing the clot while performing procedure

Term
anti-thrombin 3 test
Definition

inhibits thrombin and facters IXa,Xa,XIa,and XIIa

controls the majority of natural anticoagulation action

Term
principle for AT3 test
Definition

in the presence of heparin, thrombin is neutralized at arate that is proportional to the anti-thrombin concentration

after defibrination concentration, plasma is assayed in a 2-stage procedure that use standardized amounts: heparin,fibrinogen, thrombin

the resulting clotting time is interpeted using a calibration curve.

Term
protein s and c testing
Definition
protein c(serine protease) and s(cofactor) work in concert to slow clot formation by inhibiting coagulation factors
Term
heparin
Definition

enhances the action of AT3: acts immediately

long-term usuage deterred by: short half life, administration by intravenous or subcutaneous routes

therapy usually monitored by the activated partial thromboplastin time (aptt)

 

Term
purpose of mixing studies
Definition

mixing studies performed after a pt test where pt time is prolonged

results  of mixing study will determine if a prolonged pt is due to either: factor deficiency; inhibitor present in the patients plasma

Term
prinicipal of mixing test
Definition

normal plasma mixed with the abnormal patients sample

if the addition of normal plasma fails to correct the clotting time to a normal range: cause of the abnormal pt test is likely an inhibitor

if the pt of the mixture tests normal ad remains normal after prolonged (2 hr) incubation: results indicate the presence of one or more factor deficiencies

Term
purpose of Activated clotting time test
Definition
measures the anticoagulation affects of heparin before, during, and after surgeries that require intense coagulation measures ( i.e. open heart surgery)
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