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Coagulopathies

Hematology

QuestionAnswer
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: Adam Barnard Adam Barnard