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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

Factor 5 Leiden is what classification of disease?

thrombotic