Exam 2

Front 1

Hemophilia A

Back 1

-Mutation in genes on X link chomosome
-Inherited by sons of carrier mother
-deficiency of the factor VIII portion of the factor VIII/F complex

Front 2

Describe the specimen used for coagulation testing

Back 2

Whole blood collected by venipuncture trauma free, blue top tube, sodium citrate, 3.2%, Ratio is 1:9 1 part anticoagulant: 9 part blood. Centerfuge to remove platelets <10 X 10 9th /L platelet count leave on RBC or remove to plastic tube, Test plasma within 4 hours.

Front 3

Bleeding time

Back 3

platelet function disorders-vWillebrand's disease, bernard souiler, storage pool disease

Front 4

PT

Back 4

Test of Extrinsic pathway, Inherited or acquired defects, Effectiveness or Oral anticoagulation therapy.

Front 5

APTT

Back 5

Activated partial Thromboplastin Time, Measurement of the intrinsic Pathway, Inherited or acquired defects, monitoring heparin therapy, detect inhibitors.

Front 6

PT

Back 6

Test of Extrinsic pathway, Inherited or acquired defects, Effectiveness or Oral anticoagulation therapy.

Front 7

APTT

Back 7

Activated partial Thromboplastin Time, Measurement of the INTRISTIC PATHWAY, Inherited or acquired defects, monitoring heparin therapy, detect inhibitors.

Front 8

TT

Back 8

Qualitative measurement of thrombin clotting fibrinogen to fibrin clot, straight plasma and sensitive (weak) thrombin reagent, Normal range 10-18 seconds.

Front 9

Fibrinogen assay

Back 9

measures fibrin concentration thrombin clots the fibrin
Factor assay: used to determine the concentration of specific factor, reported in % activity as compared to a reference plasma of 100%, normal range 50-150%, factor deficient plasma, containing all factors except the one being tested, is added to dilutions of the patient plasma, tests performed and compared to reference plasma curve.

Front 10

Describe the INR and its use

Back 10

INR is international Normalized Ratio and it is developed to minimize interlaboratory variability. The PT ratio equivalent to using WHO international Referene Prepartion as the source of thromboplastin in the performance on the PT.

Front 11

Describe heparin as an anticoagulant and list the coagulation factors affected.

Back 11

Sulfated mucopolysaccharide found in the granules of mast cells and basophil granules. Isolated from the liver, binds to AT3 rapid inhibition of serine proteases.
Coagulation factors affected is factors II, VII, IX and V

Front 12

3.) For Von Willebrand’s disease, describe the pathophysiology, clinical symptoms and laboratory findings

Back 12

Quantitative or qualitative deficiency of vonWIllebrand factor, Mutations in the vWf gene.
Ridge between activated platelets and exposed collegen w/ vessel injury.
Complexes with and stabilizes Factor VII in plasma, structure is a chain of subunits multimers, produced in endothelial cells and magakaryocyte.
Bleeding symptoms are usually mild in mucosal and cutaneous tissues, epistaxis, hypermenorrhea, gingival bleeding, like those with platelet disorders.
Laboratory: Clinical history of typical bleeding symptoms, bleeding time prolonged, APTT may be prolonged; correlates w/vWf, PT, Fibrinogen normal, diagnosic is VonWillebrand antigen-immunologic-, Ristocetin cofactor assay, vWf multimers.

Front 13

4.) Characterize Disseminated intravascular Coagulation (DIC) by etiology, pathophysiology and laboratory results

Back 13

Always a secondary response to a primary disease process, alteration of hemostasis, uncontrolled fibrin formation and lysis in vessels, primary disease state initiates coagulation, systemic activation of coagulation. Injury to endothelial cells-exposing collagen activates the intrinsic pathway and platelets; infections: bacterial, viral, fungal, rickettsial, cytokines released into tissues by inflammation, activate endothelial cells, DIC seen in 30-50% of gram-negative or gram-positive septicemias, tumor necrosi-some TF like; some directly activate factor X, Tissue injury-releasing tissue thromboplastin, activating the extrinsic pathway; buns extensive surgery, snake bite, some leukemia-acute promyelocytic circulation, complications of pregnancy-abrupto placenta, retained dead fetus, amniotic fluid embolism.
Red cell and/or platelet injury releases phospholipids surface for coagulation reactions, transfusion reactions, malaria, ALE-systemic lupus erythematosus
Laboratory findings: platelet count-dec to 40-75x10 9th, PT prolonged (dec Fib, inc FDP), D dimer-increased, RBC fragments-could be present.
Treatment: Eliminate underlying cause, bleedin-factors and platelets consumed, complications of fibrin clots, acute-bleeding (80-90%) chronic thrombosis-compensated.
Replacement therapy; platelets, fresh frozen plasma, cryoprecipitate, RBC, Low molecular weight heparin.

Front 14

Describe the formation of acquired inhibitors and laboratory findings.

Back 14

Circulating anticoagulants, IgG or IgM, alloantibodies or autoantibodies, directed towards a single factor or directed against phospholipids.

Front 15

Describe the action of the Lupus anticoagulant, clinical findings, and laboratory testing.

Back 15

IgG, IgM or IgA or a mixture, inhibit 1 or more phospholipid dependant coagulation tests, APTT prolonged; mixing studies will not correct, Confimatory tests- inhibitor directed against phospholipid neutralization, patients have no clinical bleeding. 10% of SLE patients develop LA but first 2 studies had manyu SLE patients,, recurrent fetal loss, malignancies, autoimmune states, infections-viral and bacterial, drugs-procainamide, antibiotics, quinine and quinidine, damage to endothelial cells expose phospholipids that bind prothrombin and trigger antibody formation.

Front 16

Explain laboratory findings for coagulation testing in newborn.

Back 16

Platelets normal, liver is immature, fibrinogen, factor V, factor VIII-normal, contact and Vit K dep. Factors 30-60%, PT prolonged, but normal 3-4 days post Vit K, APTT prolonged, normal in 4-6 months, indwelling catheters, born to diabetic mothers, asphyxia, infection, respiratory distress syndrome, hereditary thrombophilia