Flashcards in Hubbard Deck (63)
Distinction between TTP and DIC
PT/PTT in TTP are normal early in the disease, but later tests suggest DIC
Treatment for TTP - 100% life saving
"pseudohemophilia" with mucocutaneous bleeding
Platelet aggregation test (vWF)
Osler Weber Rendeu Syndrome is defined as __ and also called ___
Also called: Hereditary Hemorrhagic Telangiectasia
-Caused by thinning of the vessel walls causing telangiectatic formations and AV malformations
Fever + neuro + thromboCP + renal + microangiopathic hemolytic anemia = ?
Thrombotic Thrombocytopenia Purpura
Hemolytic Uremic Syndrome
Define microagiopathic anemia (MAHA) and example diseases (extravascular HA)
RBCs damaged when they pass through obstructed or narrowed vessels.
-DIC, TTP/HUS, SLE, malignant HTN
-PBS = **schistocytes
Define macroagiopathic anemia and example diseases (extravascular HA).
Prosthetic heart valves and Aortic Stenosis may cause HA secondary to MECHANICAL destruction of RBCs.
Examples of infections that increase RBC destruction (extravascular HA).
Pathogenesis of Thrombotic Thrombocytopenia Purpura
- Hereditary -- ADAMTS13 gene mutation
- Acquired – autoantibodies at ADAMTS13
- “Von Willebrand factor-cleaving protease” (degrades vWF) = ADAMTS13.
-ADAMTS13 inhibition = decreased vWF degredation = increased platelet aggregation and thrombosis
Treat Hemophilia A with
Treat Hemophilia B with
CD#;ch# in hereditary hemorrhagic Telangiectasia.
Notable location of the hemorrhages.
CD105, ch9 --> codes for **ENDOGLIN**
-Hemorrhages on the vermilion border of lip and tongue
What is the most common cause of hypercoagulabilty?
Initiation of warfarin therapy because PRotein C and S are depleted prior to other factors, resulting in increased coagulablity.
Antiphospholipid syndrome is an anti/pro coagulant?
What do you use to diagnose.
***What condition is present in 50% of cases, but is NOT necessary to diagnose?
Tx with what?
-Use DRVVT (diluted Russell Viper Venom Test) or PTT test
****NOT NECESSARY to have connective tissue disease to have this.
Presence of what two factors increase risk of DVT or PT by 35-fold?
OCP + heterozygous Factor5 Leiden = synergistic relationship
Pts with vWD show up first with what symptom?
Treatment of vWD - does what? 1) cryoprecipitate 2) DDAVP
1. Cryoprecipitate - replaces vWF
2. DDAVP - causes release of vWF from endothelium
Clinical features of ____?
-Easy bleeding & bruisability
-Hematomas from bleeding into soft tissues and muscles
-Hemarthroses – frequent, many patients have 1 or 2 target joints which have recurrent bleeds
-Patients are significantly inc risk for bleeding during and after surgery
Treatment of Hemophilia A
- Factor replacement for bleeds, surgical prophylaxis
- Recombinant factor replacements – hepatitis and AIDS less of a problem
- Factor VIII inhibitors – antibodies to factor VIII that may complicate care
What deficiency can occur in patients taking warfarin, on chornic antibiotics, rat poison ingestion, or severe liver disease?
Vit K deficeincy
Characteristics of ____
o Prolonged PT
o Def of factors II, VII, IX, X, Protein C & S
o Anticoagulants = C & S
Vit K deficiency
Treatment for Vit K deficiency. Use which when?
- Vit K replacement (intact liver)
- FFP (used when liver dz or acute hemorrhage)
What is the only endothelial syndrome associated with hemostatic complications?
Hereditary Hemorrhagic Telangectasia
Clinical presentation for...
o Minimal to early death from recurrent pulmonary emboli
o Chronic leg ulcers, deep vein thrombosis, recurrent LE thrombophlebitis
o DVT inc in pregnancy
Antithrombin 3 deficiency.
tendency to clot more/hypercoagulable
Antithrombin 3 deficiency treatment
-Prophylactic treatment with anticoagulants
-Patients with DVT should receive heparin but in higher doses
-AT-III replacement therapy, if don’t respond to heparin
Action of protein C
inactivates Factors 5 and 8
Use what in Protein C deficiency to decrease risk of thromboembolic disease?
What is antithrombin 3? What amplifies it?
serine protease that inhibits activation of thrombin.
-Heparin amplifies activity
-In absence of antithrombin3, hypercoagulablity
Most common cause of hypercoagulable state from deficiency in Protein C or S
initiation of warfarin therapy