Bleeding disorders

Question 1

4 steps in stopping bleeding

Answer 1

1. promary hemostasis - platelets and VWF
2. secondary - coagulation cascade
   - generate thrombin
   - form fibrin clot
3. clot stabilization
   - factor 13 and thrombin activator
4. healing

Question 2

what is center of cascade and rate limiting step

Answer 2

factor 10

Question 3

what is common pathway

Answer 3

10+5> 2 (prothrombin (2) becomes thrombin (2a)> fibrinogen to fibrin (clot)

Question 4

what is path of extrinsic

Answer 4

7+tissue factor to activate 10

Question 5

what is intrinsic pathway

Answer 5

HMWK and prekallikrein>12>11>9+8>10

Question 6

what are contact factors

Answer 6

• prekallikrein
• HMWK
• Factor 12
  NOT needed to control bleeding

Question 7

what are 2 tests and what do they test?

Answer 7

1. protrombin time (INR) - extrinsic and common

2. activated partial thromboplastin time (PTT) - int and extrinsic

Question 8

what is INR and what is is designed for

Answer 8

standardized PT

- measure for warfarin

Question 9

2 general types of coagulation factors

Answer 9

1. non-enzymatic types
   - 5, 8, and TF
2. zymogens
   - 2,7,9,10 - all K dependend

Question 10

where are most factors made and 4 exeptions

Answer 10

liver

1. tissue plasminoggen activator (tPA)
2. VWF
3. thrombomodulin
4. 8 is made in liver but also endothelium

Question 11

what is factor with shortest half life

Answer 11

7, then 5+8

Question 12

where does coag occur

Answer 12

on membranes with expose phospholipids

- not resting cells

Question 13

what is 1 inherited disorder of primary hemostasis

Answer 13

von willbrand disease

Question 14

what is 2 inherited disorder of secondary hemostasis

Answer 14

1. hemophilia

2. 11 def.

Question 15

what is 3 acquired disorder of primary hemostasis

Answer 15

1. drugs!
2. thrombocytopenia
3. uremia

Question 16

what is 4 acquired disorder of secondary hemostasis

Answer 16

1. DRUGs
2. liver dis
3. DIC
4. vit K def

Question 17

what is best predictor of bleeding disorder

Answer 17

bleeding Hx

Question 18

what is VWF

Answer 18

• synthesized in endothelial cells

- platelet adhesion(to wall) and aggregation (to each other)

Question 19

3 clinical components of VW disease

Answer 19

1. bleeding
2. fam Hx
3. lab results

Question 20

2 types of VW bleeds

Answer 20

1. mucocutaneous
   - menorhhagia
   - epistaxis
   - bruiding
2. musculoskeletal

Question 21

what is familial pattern

Answer 21

autosomal inheritance

Question 22

3 classes of VW disease

Answer 22

1. mild/moderate quant. traits
2. qualitative traits
3. severe qual traits

Question 23

4 lab findings in VW dis.

Answer 23

1. normal CBC and PTT/INR
2. low VWF antigen
3. low VWF cofactor
4. factor 8 activity decreased

Question 24

treatments of VW dis.

Answer 24

medical
- desmopressin
- transfusions
- OCP
- topic agents
gyne
- hormonal IUD
- endo ablation

Question 25

2 types of x-linked hemophilia

Answer 25

B - factor 9 | A - factor 8

Question 26

Sx of hemophilia

Answer 26

- MSK bleeds - mouth bleeds, epistaxis - IC bleeds - trauma bleeds

Question 27

principle of bleed Mgmt

Answer 27

- treat first | - investigate later

Question 28

4 categories of treatment

Answer 28

1. specific replacement of factors 2. topical agents to promote fibrin clot formation 3. anti-fibrinolytic agents 4. RICE

Question 29

what is effect of liver disease

Answer 29

can get both bleeds and clots

Question 30

5 effects of liver disease

Answer 30

1. less synthesis - except 8 2. abbreant synthesis 3. decreased clearance 4. accel. comsumption 5. impaired fibrin formation

Question 31

5 clues to liver based issues

Answer 31

1. thrombocytopenia 2. prolonged PT/INR 3. decreased factors excepy 8 4. fibrinogen assay 5. DIC

Question 32

3 treatments for liver disease

Answer 32

1. treat liver disease 2. vit K 3. if major - desmopressin, anti-fibrinogen

Question 33

what is DIC

Answer 33

- aquired syndrome of widespread coag - overwhelms normal anti-thrombotic mechs - may result in systemic bleeding or clots

Question 34

patho of DIC

Answer 34

uncontrolled creation of thrombin and plasmin > intravascular fibrin > consumption of all hemostasis products > activation of lysis

Question 35

conditions assoc. with DIC

Answer 35

excessive TF release - sepsis - trauma - CA - obstetrical - vascular - immune - toxins/drugs

Question 36

test for DIC

Answer 36

none | - clinical + abnormal hemo screening

Question 37

treat of DIC

Answer 37

treat underlying cause

Question 38

what is vit K funciton

Answer 38

facilitates carbolxylation of 6 factors (10,9,7,2,C,S)

Question 39

5 causes of vit K def.

Answer 39

1. warfarin 2. poor intake 3. ABs 4. biliary obstruction 5. hemmoragic disease of newborn

Question 40

3 lab findings in vit K def.

Answer 40

1. long IRN 2. long PTT 3. low factos 10,9,7,2

Question 41

what is DDx for long PTT but normal PT

Answer 41

1. single factor def. (inheritied) 2. VW disease 3. inhibirots - aquired - drug (hepain) -

Question 42

DDx for long PT, but normal PTT

Answer 42

inherited or aquired factor 7 def.

Question 43

DDx for long PTT and PT

Answer 43

1. inherited def. of common pathway 2. inherited abnormal fobrinogen 3. aquired - inhibitors - vit K def - too much warfarin - servere liver - DIC