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Coagulopathies
Hematology
| Question | Answer |
|---|---|
| Causes of vessel wall dz | Scurvy (Vit C def); Amyloidosis; Hereditary-Hemorrhagic Teleangiectasia (Osler-Weber-Rendu Dz); Neurofibromatosis; Collagen Dz (Marfan; Ehler-Danlos) |
| micro-angiopathic hemolytic anemia (MAHA) lab findings | TTP; anemia, hemolysis, schistocytes |
| Extrinsic & intrinsic coag pathways converge to form: | factor Xa |
| All clotting factors (except FVIII) are made in: | Liver |
| Effects of liver dz on coagulation | 1st: decrease in factor VII (short half-life) prolongs PT; then decrease in other factors -> prolonged aPTT. Disease causes splenomegaly -> thrombocytopenia |
| MOA: warfarin tx on high PT | Warfarin antagonizes the action of Vitamin K |
| Vitamin K is a necessary co-factor in the g-glutamyl carboxylation of: | Factors II, VII, IX, X (these along with Ca & platelets activate FX & FII in coag cascade). Also Protein C and Protein S |
| MOA of vitamin K deficiency on high PT | Similar to MOA of warfarin |
| Deficiencies in HMWK, PK, and FXII are assoc w/which bleeding problems? | Deficiencies in HMWK, PK, and FXII are NOT associated with any bleeding |
| Clotting factor def assoc w/Jewish ancestry | FXI def |
| Factor VIII deficiency AKA: | Hemophilia A |
| Factor IX deficiency AKA: | Hemophilia B |
| FVIII & FIX deficiency: percentages associated with bleeding & severity | <1% assoc w/ spontaneous bleeding. 1-5% moderate, can still have spontaneous bleed. 5-40% can bleed w/procedures or trauma |
| Hx includes hemarthrosis, bleeding into joints, and/or x-linked FH of problems: | Factor IX deficiency; FVIII deficiency |
| FVIII & FIX deficiency findings | Labs: aPTT prolonged; FVIII/FIX levels decreased |
| FVIII & FIX deficiency mgmt | Specific factor replacements: FVIII or FIX concentrate (plasma derived or recombinant). Avoid IM injections or meds causing bleeding. |
| FVIII Inhibitor | Seen in PG & the elderly; Presents with provoked – spontaneous bleeding; Rare ( 1:1,000,000); Auto-Ab vs FVIII, prevents fn or increasing clearance |
| Treatment to Eradicate FVIII Inhibitor: | Steroids +/- Cyclophosphamide. Tx if Bleeding: If inhibitor is of low titer, can give FVIII; if titer is high, give FVIIa |
| limited to mucocutaneous bleeding | vW Dz |
| Functions of von Willebrand factor | Bridges platelets together and with endothelium. Carrier for FVIII, extending its half-life |
| von Willebrand disease tx (for mild forms): | Avoid aspirin. DDAVP (synthetic vasopressin), intranasal or IV -> increase release of pre-formed vWF (only works for a day or two). e-aminocaproic acid works by inhibiting fibrinolysis |
| von Willebrand disease tx (for Severe Bleeding/Surgery): | Replace with VWF:FVIII containing concentrates; Humate-P. DDAVP pre-op? |
| Coagulation dz: causes elevated levels of prothrombin; leads to a modest increase in hypercoagulability, (2-fold) | Prothrombin 20210A Mutation |
| Second most common inherited cause of hyper-coaguability | Prothrombin 20210A Mutation |
| Prothrombin 20210A Mutation: concomitant risk factors | Smoking, BPCs/Hormone replacement |
| Fibrinolysis/Anti-coagulant Disorders usu present as: | thrombosis |
| Antithrombin activity is markedly stimulated by: | Heparin |
| Antithrombin def is manifested by: | Recurrent venous thromboses |
| Protein C or Protein S Def: Predisposition to: | recurrent venous thromboses |
| Uncommon causes of venous thromboses | Antithrombin def; Protein C or Protein S Def |
| Disease in which mutation causes resistance to inactivation by Activated Protein C | Factor V Leiden |
| Most common hereditary hyper-coagulable state; mainly limited to ? | Factor V Leiden; pts of Euro extraction |
| Factor V Leiden: Heterozygous state increases risk of thrombosis up to: | 8-fold |
| Factor V Leiden: Homozygous state increases risk: | 100-fold |
| Auto-antibody that interferes with the natural anti-coagulant system | Anti-Phospholipid Syndrome |
| Anti-Phospholipid Syndrome presents with: | Recurrent arterial or venous thromboses; 2nd trimester miscarriages; Thrombocytopenia; Valvular heart dz |
| Anti-Phospholipid Syndrome: Lab Data: | Presents w/prolonged aPTT (b/c in vitro fx of the Ab on aPTT); mixing study fails to normalize; Confirmatory tests required to make the diagnosis |
| Anti-Phospholipid Syndrome: tx | Once thrombotic event occurs, tx w/warfarin indefinitely |
| Condition in which circulating plasma (which contains clotting factors) turns into serum | DIC |
| DIC: presentation | Excessive thrombosis (small-midsize vessels: organ infarcts); organ dysfunction (renal; hepatic; CNS: hematuria, GIB, petechiae); bleeding, shock, death |
| Acral ischemia seen in: | DIC |
| Hx pos for heparin, tacrolimus, ticlopidine may point to: | DIC |
| Signs of hemorrhage in DIC | Mucous membranes; IV Sites, catheters, ETT; venipuncture sites |
| DIC tx (if bleeding, or surgery needed) | Platelets. PRBCs. Fibrinogen if <100 (Give cryo: worsen thrombosis if given alone, as cryo lacks AT and proteins C & S) |
| Defn Hemorrhage: | failure to maintain hemostasis |
| Defn Thrombosis: | failure to maintain fluidity |
| Steps in forming the primary hemostatic plug: | Platelet adhesion; platelet aggregation; platelet surface activation & recruitment of other platelets; platelet release |
| Thromboxane A2 is: | a potent stimulator of platelet aggregation & vasoconstriction |
| Visible end product of coagulation = | Fibrin |
| DIC tx | Treat underlying condition. Replace blood products PRN (platelets, FFP, cryo for fibrinogen, PRBCs, ATIII). Possibly heparin or LMWH |
| Most common congenital disorder of hemostasis | von Willebrand (prevalence 1%) (2nd: hemophilia A) |
| von Willebrand genetics | autosomal dominant |
| von Willebrand factor is carrier protein for: | factor VIII. vWF patients have decreased factor VIII levels & prolonged PTT |
| Glanzmann syndrome = | rare autosomal recessive. Deficiency in factors IIb/IIIa; platelets cannot aggregate. High bleeding time. |
| Tx for Glanzmann syndrome | platelet transfusion |
| Liver is the site of production of all clotting factors except: | factor VIII |
| Thrombopathies | Von Willebrand. Glanzmann thrombasthenia. Bernard-Soulier. Acquired |
| Tx for liver dz/thrombocytopenia | FFP +/- cryoprecipitate (contains concentrated fibrinogen) |
| Causes of acquired thrombopathy | Drugs (ASA, NSAIDS, cephalosporins); ETOH (>BT, >*PT, PTT also prolonged in end-stage liver dz); Renal dz (unknown mechanism); Dysproteinemias or MM |
| Defn Thrombopathy (Qualitative) | Bleeding disorders characterized by prolonged BT despite normal platelet count |
| Subacute DIC (Trousseau syndrome): will see: | Recurrent superficial & DVTs; low platelet & high D-dimer |
| Natural anticoagulants | Proteins C & S |
| Causes of vitamin K deficiency | biliary tract disease, drugs (Abx, warfarin), malnutrition, malabsorption |
| Fibrinogen unique to: | plasma; ref range = 206-382 |
| Factor Xa to monitor: | LMWH |
| Hemophilia A & B clinical features | Hemarthroses (knees, ankles, elbows). Arthralgias. Low FVIIIc; high PTT. |
| Hemophilia B clinical features | Christmas disease. X linked recessive; males; low FIX; high PTT. Tx = Factor IX, NO DDAVP, NO ASPIRIN |
| Lupus Anticoagulant | Circulating IgG or IgM; high PTT (no correction with mixing studies); risk of thrombosis or recurrent spontaneous abortion |
| Most frequent cause of hereditary thrombophilia | Factor V Leiden (AKA APC); hereditary resistance to action of activated protein C (4-8% of pop) |
| Diff btw hep & LMWH | Hep binds to anti-thrombin III (inactivate coag factors); LMWH binds to ATIII and directly inactivates Xa |
| D-Dimer | Nonspecific; only neg test is helpful; PE; 90% pts w/thrombotic & thromboembolic dz; Neg <0.349; Low Pos = 0.350-0.500; Positive = >0.500 |
| Protein C def pts | warfarin hyper-sensitivity rxn; may have to decrease coumadin by half (lest get necrotizing condition) |
| ACT | Activated clotting time; monitors heparin in OR (check if safe to pull cath/A lines); Normal blood clots in <100 seconds; Heparin therapeutic = 300-600 s |
| Virchow's Triad | Vascular damage; Hypercoagulability; Vascular Stasis; Predisposes patient to venous thrombosis |
| Distinguish liver disease coagulopathy from vitamin K deficiency | Vitamin K deficiency only: 5 is normal, 7 is low. Liver disease only: both 5 & 7 are low |
| Spontaneous hemarthrosis may be sx of: | Hemophilia A |
| Mucosal or gingival bleeding, epistaxis, menorrhagia | Von Willebrand dz |
| Continuous bleeding post-op or trauma; s/p multiple blood transfusions; low platelets, increased PT, INR, + fibrin split products (increased D-dimer): | DIC |
| Coag pathways | Intrinsic 12, 11, 9, 8; extrinsic 7; common 1,2,5, 10 |
| thrombopathy genetics | von W auto dominant. Glanzmann and Bernard-Soulier auto recessive |
| Trousseau syndrome = | subacute DIC in cancer pts |
| hemophilia C | factor XI: x linked recessive, Ashkenazi; long PTT |
| Vit K-dependent factors | 2, 7, 9, 10 |
| heritable hypercoagulable states | Factor V Leiden; pro C & S deficiency; antithrombin III deficiency |
| Hereditary resistance to action of activated protein C = | Factor V Leiden (APC resistance) |
| Deficiency allows unopposed conversion of fibrinogen to fibrin = | Antithrombin III deficiency |
| giant plts; auto rec; no von W factors | Bernard-Soulier dz |
| Most common severe bleeding disorder | hemophilia A (low VIIIc) |
| Hemophilia C (factor XI def) tx | FFP |
| Von Willebrand Dz tx: | ddAVP (synthetic vasopressin); avoid aspirin |
| Most common congenital coagulopathy | von Will (1% prevalence) |
| Vitamin K deficiency is seen in: | HDN (tx with vitamin K). Malabsorption (of fat soluble vitamins: A,D,E, K). Malnutrition. Abx use |
| Recurrent arterial or venous thromboses; 2nd trimester miscarriages; thrombocytopenia; valvular heart dz; suggests: | Anti-Phospholipid Syndrome |
| fibrin is digested by | plasmin |
| vWF is synthesized in: | endothelial cells & megakaryocytes |
| Function of vWF: | mediate platelet adhesion to damaged vessel site |
| Bernard-Soulier Syndrome | Rare autosomal rec; low/abn plt vWF receptor; giant plts, thrombocytopenia, prolonged BT |
| The ___ pathway initiates coag process; then _____ pathway enhances _____ formation in clot | Extrinsic; intrinsic; thrombin |
| First factors to be affected in hepatic dysfunction | Vit K dependent factors (2, 7, 9 & 10) + factor 5 |
| DIC pathology | Consumption of coag factors 2/2 intravascular activation of coag process with secondary activation of fibrinolysis -> hemorrhage or thrombosis |
| Common causes of DIC | Infectio (often GNR endotoxins), PG complications, massive tissue injury, hypoxia, acidosis, malignancy, snakebite, acute promyelocytic leukemia (M3 variant of AML) |
| Hemophilia A epidemiology | X linked recessive: Males. 1 in 10,000 live births. 80% of hemophilia patients have Hemo A. |
| Hemophilia B epidemiology | X linked recessive: Males. 1 in 30,000 live births. |
| Hemophilia A mgmt | Factor VIII. DDAVP (transient rise in FVIII, leads to tachyphylaxis) |
| Portal HTN can lead to: | splenomegaly, mild thrombocytopenia, varices, PUD, GI bleed |
| Antithrombin III (ATIII) deficiency pathology | ATIII inactivates surplus thrombin. Activity potentiated by heparin. ATIII deficiency (AD or acquired) -> venous thrombosis |
| ATIII deficiency mgmt | If VTE: IV heparin -> PO warfarin indefinitely |
| Protein C deficiency pathology | Protein C = vit K-dependent liver protein to stimulate fibrinolysis & clot lysis. Deficiency -> recurrent VTE & PE. |
| Protein C deficiency clinical features | Warfarin-induced skin necrosis. Purpura fulminans or fulminant intravascular coagulation in newborns. Possibly CVA. |
| Protein C deficiency mgmt | IV heparin -> PO warfarin indefinitely |
| What % of patients with DVT/PE have cancer? | 15% (another 5% without cancer dx will have cancer dx within 1 year) |
Created by:
Abarnard
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