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

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

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

abnormally high plt ct: >450,000

can cause strokes due to plt clots

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

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

prolonged bleeding time

plt count is normal to slightly elevated

aptt may be abnormal

pt is normal

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

contains all coagulation factors normally found in the circulating blood

treatment for coagulations factors disorders

contains vwf, fibrinogen, factorsVII and XIII

factor VIII concentrate and ATIII concentrate

treatment for coagulation factor disorders

hereditary deficiencies:

hypofibrinogenemia

dysfibrinogenemia

impaired production

excess utilization

fribinogen destruction

complication of surger

plasma fibrinogen less than 100mg/dL

no major bleeding

prolonged PT, APTT, and Thrombin time

 treat with cyroprecipitate and FFP

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

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

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

inherited as a sex-linked recessive trait

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

also known as christmas disease

congenital deficiency

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

normal plt ct, bleeding time, and pt

prolonged APTT

Treatment: ffp, factor XI complex, factor XI concentrates

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

acquired deficiency: liver disease, vitamin k deficiency, coumadin

rare

lab results: prolonged pt and aptt

congenital deficiency

bleeding less severe than hemophilia a and spontaneous hemorrhaging is rare

lab results: normal pt, prolonged aptt

most frequently occuring inhibitor

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

bleeding is uncommon, thromobosis in 30% of patients

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

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

decreased level of vitamin K dependant factors

decreased factor v and the contact factors

abnormal fibrinolysis

thrombocytopenia and qualitative platelet disorders

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

a pathological form of coagulation that is diffused rather than localized

characterized by generalized bleeding and shock

increased pt and aptt

decreased fibrinogen and plt ct

positvie fdp and d-dimer

fragmented rbcs in peripheral

treatment: eliminate underlying disorder

large platelets greateer in size than a normal rbc

associated with disease states having increased rate of platelet production

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

fibrinogen level below 100 mg/dL

impaired fibrinogen function

fibrin degradation products

thrombolytic agents used to monitor streptokinase and urokinase therapy

purpose: measures concentration of fibrinogen (factor I)

reference  range: 220- 498 mg/dL

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

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

 decreased: dic,congenital afibrinogenemia, hypofibrinogenemia

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

reference range: <10ug/ml (adult)

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

primary fibrinolysis

dic with secondary fibrinolysis

thrombosis states

alcoholic cirrhosis of the liver

late pregnancy

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

indicates that a stable fibrin clot has lysed

reference range: 2-6ng/dL

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

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

dic with secondary fibrinolysis

sickle cell disease

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

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

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

controls the majority of natural anticoagulation action

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.

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)

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

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