Flashcards in Hubbard Deck (63)
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1
Distinction between TTP and DIC
PT/PTT in TTP are normal early in the disease, but later tests suggest DIC
2
Treatment for TTP - 100% life saving
plasmapheresis
3
"pseudohemophilia" with mucocutaneous bleeding
Factor8-vWD
4
Platelet aggregation test (vWF)
ristocetin
5
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
6
Fever + neuro + thromboCP + renal + microangiopathic hemolytic anemia = ?
Thrombotic Thrombocytopenia Purpura
Hemolytic Uremic Syndrome
7
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
8
Define macroagiopathic anemia and example diseases (extravascular HA).
Prosthetic heart valves and Aortic Stenosis may cause HA secondary to MECHANICAL destruction of RBCs.
-PBS=schistocytes
9
Examples of infections that increase RBC destruction (extravascular HA).
Malaria, babesia
10
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
11
Treat Hemophilia A with
Treat Hemophilia B with
Factor 8
Factor 9
12
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
13
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.
14
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?
PROcoagulant
-Use DRVVT (diluted Russell Viper Venom Test) or PTT test
****NOT NECESSARY to have connective tissue disease to have this.
Tx: hydroxychloroquine
15
Presence of what two factors increase risk of DVT or PT by 35-fold?
OCP + heterozygous Factor5 Leiden = synergistic relationship
16
Pts with vWD show up first with what symptom?
mucocutaneous bleeding
17
Treatment of vWD - does what? 1) cryoprecipitate 2) DDAVP
1. Cryoprecipitate - replaces vWF
2. DDAVP - causes release of vWF from endothelium
18
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
Hemophilia A
19
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
20
What deficiency can occur in patients taking warfarin, on chornic antibiotics, rat poison ingestion, or severe liver disease?
Vit K deficeincy
21
Characteristics of ____
o Bleeding/hemorrhage
o Prolonged PT
o Def of factors II, VII, IX, X, Protein C & S
o Anticoagulants = C & S
Vit K deficiency
22
Treatment for Vit K deficiency. Use which when?
- Vit K replacement (intact liver)
- FFP (used when liver dz or acute hemorrhage)
23
What is the only endothelial syndrome associated with hemostatic complications?
Hereditary Hemorrhagic Telangectasia
24
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
25
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
26
Action of protein C
inactivates Factors 5 and 8
27
Use what in Protein C deficiency to decrease risk of thromboembolic disease?
warfarin
28
What is antithrombin 3? What amplifies it?
serine protease that inhibits activation of thrombin.
-Heparin amplifies activity
-In absence of antithrombin3, hypercoagulablity
29
Most common cause of hypercoagulable state from deficiency in Protein C or S
initiation of warfarin therapy
30
