Week 9 Flashcards

Question

FXIII deficiency

Answer 1

-rare -happens in 3 forms all related to FXIII structure -PT, APTT and TT – Normal -pt can form weak clots (non cross linked) that dissolve REMEMBER FXIII IS ALSO FIBRIN STABILIZING FACTOR -do reflex testing - 5M Urea testing , FXIII quantitative

Answer 2

-fatal hemorrhage in trauma related deaths -triggered by systemic shock -leads to Coagulopathy and hemorrhage by ADAMS13 reduction, TF release, Coaf factor activation, hyperfibrinolysis and loss of coag control proteins treatment: Massive Transfusion (RBC Infusion) + Plasma (Thawed plasma or FP-24) + PLT concentrate (1:1:1- in equal amounts - approximating makeup of whole blood)

Answer 3

- bleeding because of liver disease can be localized or generalized, mucocutaneous or anatomic -Mucocutaneous bleeding - associated with thrombocytopenia and decreased platelet function -Anatomic or soft tissue bleeding occurs because of procoagulant dysfunction and deficiency -symptoms are jaundice and abdominal tenderness

Answer 4

-liver produces factors and coag regulatory proteins -if hepatocytes are damaged then factors are decreased -VWF is an exception – VWF is produced by megakaryocytes and endothelial cells -FVIII also an exception – is also produced by vascular endothelial cells -Vitamin K - dep factors also affected (FII, VII, IX, and X, and control proteins C, S, and Z) -they are released without a 2nd carboxyl group and cannot participate in coag -FVII affected first because it has the shorted half life

Answer 5

PT -prolonged -sensitive to FVII activity, it is a early marker for liver disease APTT -prolonged in severe disease Plt count -mild thrombocytopenia Fib -increased at first then reduced/abnormal later in disease as the disease severity increases

Answer 6

-Non-vitamin K-dependant, labile factor -Reduced in liver disease (as well as FVII) -Assessed by FV assay -Not reduced in dietary Vitamin K deficiency -Helps to differentiate the two diseases

Answer 7

-Elevated in early liver disease - because is an APR -Moderate or severe liver disease -Dysfibrinogenemia occurs – fibrinogen is coated with sialic acid- leading to poor fibrinogen function and resulting in general anatomic bleeding -Marked decrease in end-stage liver failure -Fibrinogen levels severely decreased (hallmark)

Answer 8

Platelet Abnormalities -moderate thrombocytopenia because of shortened PLT survival, low production -seen in reduced PLT aggregometry testing -cause would be portal hypertension and hepatosplenomegaly Systemic Fibrinolysis -diseased liver does not clear circulating plasmin which is a fibrinolytic serine protease

Answer 9

Oral or IV Vitamin K therapy- short lived because liver metabolism is impaired Transfusion of thawed Frozen Plasma, collected from normal donors - with ALL coaf factors Cryoprecipitate in severe cases (Fibrinogen <0.5 g/L= bleeding is imminent) - VERY CONCENTRATED FIB Liver transplant in severe cases of the disease

Answer 10

-uncontrolled hemostasis activation secondary to disease process -causes both bleeding and clotting because platelets and coagulation factors are being used up at a fast rate -CONSUMPTIVE COAGULOPATHY -affects primary hemo (plt decrease), and 2ndary hemostasis (factor consumption = increase in PT APTT) -causes clot formation in small vessels -sets up systemic toxic and inflammatory processes that decrease flow to tissues, organs -moderate to life threatening -Acute is fatal and DIC can be chronic

Answer 11

DIC -low production of regulatory proteins (AT, Protein C and S) -release of activated procoag from degenerating hepatocytes that cant be cleared by the liver

Answer 12

Lab results -Platelet count decreased -PT, APTT and TT are all prolonged -Fibrinogen level may be decreased -D-Dimer positive PBS Morphology -Intravascular fibrin production – Microangiopathic hemolytic anemia (MAHA) - with thrombocytopenia and schistocytes

Answer 13

Transfusion with : Thawed frozen plasma: with ALL coag factors Cryo - Concentrates FIB, FVII, VWF Platelet or RBC transfusion Heparin slows thrombotic process Routine tests to monitor therapy -PT, APTT, Fibrinogen, D-dimer, CBC (RBC Morph/PLT estimate), and Heparin assay

Answer 14

-chronic renal failure associated with PLT dysfunction causing mucocutaneous bleeding -altered plt function is the cause of bleeding -adhesion and aggregation suppressed because of antiplatelet effects of compounds like guanidinosuccinic acid or Phenolic compounds coating PLT (PRIMARY affected) -Thrombocytopenia & Anemia contribute to bleeding -PT/APTT results are normal - -2º hemostasis typically unaffected -Treatment - Dialysis, RBC transfusions, or EPO therapy

Answer 15

-found in food - leafy greans -bowel flora produces Vit K -needed daily body stores are limited caused by : -AB use -malabsorption -fad diets or IV nutrition -hemorrhagic disease of the newborn , breastfeeding delays establishment of gut flora -Coumadin overdose common in lab resulting in lack of function in VitK dep factors results in -mod to severe bleeding -if Coumadin overdose - oral Vit K therapy 3 hours -transfusion on frozen plasma

Answer 16

Factors affected II, VII, IX, X (& Protein C, S, and Z) PT PT is prolonged much before APTT Due to short ½ life of FVII - first to decrease APTT APTT is eventually prolonged (deficiency must be severe before the APTT will be prolonged) Fibrinogen Fibrinogen levels are normal or slightly decreased

Answer 17

-acquired autoantibodies have been found in non hemo pt and can inhibit FII (prothrombin), V, VIII, IX, XIII, and VWF -Autoantibodies against FVIII are most common -autoanti-FVIII is diagnostic of acquired hemophilia, seen in pt over 60 with no underlying issues causing severe anatomic bleeding into soft tissue -autoAB for other procoags are rare

Answer 18

Rheumatoid arthritis (RA) Inflammatory bowel disease Systemic lupus erythematosus (SLE) Lymphoproliferative disease Pregnancy (acquired hemophilia triggered 2-5 months after delivery)

Answer 19

-if there is sudden hemorrhage that looks like acquired hemophilia - PT, APTT, and TT ordered -If FVIII inhibitor present (most common inhibitor) - PT and TT- normal (most likely) APTT- prolonged Reflex with Mixing Study and Factor/Inhibitor assay FVIII assay - likely to show FVIII activity of 40% or less

Answer 20

-mod to severe mucocutaneous bleeding -can be suspected in ANY pt with recent bleeding with no bleeding history -Symptoms similar to congenital VWD Bleeding caused by either: Decreased VWF production Adsorption of VWF to abnormal cell surfaces OR Specific VWF Autoantibody Can be associated with: Hypothyroidism Autoimmune disorders Lymphoproliferative and Myeloproliferative disorders Intestinal disorders Congenital heart disease Pesticide exposure Uremia

Answer 21

PT – Normal (not affected) APTT - Prolonged (moderately) - VWF available to support FVIII As in congenital VWD, diagnosis based on VWF:RCo : VWF:AG ratio -Diminished VWF activity and VWF antigen levels

Answer 22

-autoantibody immunoglobulins that bind protein-phospholipid complexes (IgM or IgG isotypes) Includes antibodies- Lupus anticoagulant Anticardiolipin Anti-β2-GPI Anti-DNA -Non-specific inhibitors -manifests are unexplained venous or arterial thrombosis, thrombocytopenia or recurrent fetal loss

Answer 23

-Most common APA -found in 50% of pt with SLE (Systemic Lupus Erythematosus) -antibodies promote abnormal clotting in vivo - THROMBOTIC -Blocks or binds phospholipid in the PT/APTT reagent – preventing clotting in vitro (non-specific inhibitor)

Answer 24

-APTT prolonged -Mixing studies (APTT remains prolonged or Mix not corrected) Lupus anticoagulant profile- Dilute Russell Viper Venom Test (DRVVT) -most specific LAC assay -based on ability of venom of Russell viper to induce thrombosis -coag in venom activates FX which activates prothrombin to thrombin in presence of FV and phospholipid -LAC AB interfere with clot promoting of phospholipid in vitro causing prolonged clotting time

Answer 25

Hypofibrinogenemia - FIB level less than 1 g/L Afibrinogenemia - absence of fibrinogen Dsyfibrinogenemia - presence of FIB that is biochemically abnormal and non-functional

Answer 26

-Consequence of immune response to UFH and LMWH -Results in reduced PLT count via PLT activation, inflammation, and thrombosis

Answer 27

CBC HGB, HCT, PLT count, and RET -can show anemia from bleeding and BM response -normal in inherited factor deficiencies - abnormal in acquired disorders -Depends on underlying cause of disorder PT or APTT or both are prolonged Thrombin time (qualitative analysis of FIB activity) -If both PT & APTT are abnormal – common pathway? -Start with Fibrinogen (FI) deficiency Can be affected by inhibitors: -Heparin, DTIs (sample contamination or therapy) -Fibrinogen/fibrin degradation products -Non-specific Inhibitors (LAC) -Specific Factor Inhibitors/Antibodies Fibrinogen assay (quantitative) Platelet Function Tests - specific to confirm disease Factor Assays - to determine what factor is deficient and % activity

Answer 28

-performed on plasma used to distinguish factor deficiencies from factor inhibitors Differentiate between: Factor deficiencies (single or multiple) OR Specific Factor Antibodies or Inhibitors OR Non-specific Antibodies or Inhibitors -normal PT or APTT need 50% of normal levels in circulation -mixing studies help to determine next steps to diagnose cause of abnormal PT and APTT

Answer 29

-When fibrinogen and an inhibitor have been ruled out, prolonged result(s) are the result of single factor OR multi-factor deficiency -FACTOR ASSAYS determine which factor(s) are deficient FVII and FVIII are most common -Only PT or APTT Prolonged - specific assay -Both PT & APTT Prolonged- factors from common pathway

Answer 30

-assess FXIII deficiency or inhibition -FXIII is insoluble in urea -without FXIII, fibrin clot will dissolve rapidly in presence of urea -Positive results should be confirmed by FXIII assay Quantitative FXIII activity assay -clots PPP with CaCL2 -visual inspection for clot at 1, 2, 3, 4, and 24 hours clot at 24hrs = Normal FXIII levels No clot at 24hrs = Abnormal FXIII levels a decrease in clot size, fragmentation, cloudiness which is a FXIII abnormality -abnormal at less than 2 %

Week 9 Flashcards

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