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Bleeding disorders Flashcards

1
Q

4 steps in stopping bleeding

A
  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
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2
Q

what is center of cascade and rate limiting step

A

factor 10

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3
Q

what is common pathway

A

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

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4
Q

what is path of extrinsic

A

7+tissue factor to activate 10

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5
Q

what is intrinsic pathway

A

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

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6
Q

what are contact factors

A
  • prekallikrein
  • HMWK
  • Factor 12
    NOT needed to control bleeding
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7
Q

what are 2 tests and what do they test?

A
  1. protrombin time (INR) - extrinsic and common

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

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8
Q

what is INR and what is is designed for

A

standardized PT

- measure for warfarin

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9
Q

2 general types of coagulation factors

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

where are most factors made and 4 exeptions

A

liver

  1. tissue plasminoggen activator (tPA)
  2. VWF
  3. thrombomodulin
  4. 8 is made in liver but also endothelium
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11
Q

what is factor with shortest half life

A

7, then 5+8

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12
Q

where does coag occur

A

on membranes with expose phospholipids

- not resting cells

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13
Q

what is 1 inherited disorder of primary hemostasis

A

von willbrand disease

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14
Q

what is 2 inherited disorder of secondary hemostasis

A
  1. hemophilia

2. 11 def.

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15
Q

what is 3 acquired disorder of primary hemostasis

A
  1. drugs!
  2. thrombocytopenia
  3. uremia
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16
Q

what is 4 acquired disorder of secondary hemostasis

A
  1. DRUGs
  2. liver dis
  3. DIC
  4. vit K def
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17
Q

what is best predictor of bleeding disorder

A

bleeding Hx

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18
Q

what is VWF

A
  • synthesized in endothelial cells

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

19
Q

3 clinical components of VW disease

A
  1. bleeding
  2. fam Hx
  3. lab results
20
Q

2 types of VW bleeds

A
  1. mucocutaneous
    - menorhhagia
    - epistaxis
    - bruiding
  2. musculoskeletal
21
Q

what is familial pattern

A

autosomal inheritance

22
Q

3 classes of VW disease

A
  1. mild/moderate quant. traits
  2. qualitative traits
  3. severe qual traits
23
Q

4 lab findings in VW dis.

A
  1. normal CBC and PTT/INR
  2. low VWF antigen
  3. low VWF cofactor
  4. factor 8 activity decreased
24
Q

treatments of VW dis.

A 
medical
- desmopressin
- transfusions
- OCP
- topic agents
gyne
- hormonal IUD
- endo ablation
25
2 types of x-linked hemophilia
B - factor 9 | A - factor 8
26
Sx of hemophilia
- MSK bleeds - mouth bleeds, epistaxis - IC bleeds - trauma bleeds
27
principle of bleed Mgmt
- treat first | - investigate later
28
4 categories of treatment
1. specific replacement of factors 2. topical agents to promote fibrin clot formation 3. anti-fibrinolytic agents 4. RICE
29
what is effect of liver disease
can get both bleeds and clots
30
5 effects of liver disease
1. less synthesis - except 8 2. abbreant synthesis 3. decreased clearance 4. accel. comsumption 5. impaired fibrin formation
31
5 clues to liver based issues
1. thrombocytopenia 2. prolonged PT/INR 3. decreased factors excepy 8 4. fibrinogen assay 5. DIC
32
3 treatments for liver disease
1. treat liver disease 2. vit K 3. if major - desmopressin, anti-fibrinogen
33
what is DIC
- aquired syndrome of widespread coag - overwhelms normal anti-thrombotic mechs - may result in systemic bleeding or clots
34
patho of DIC
uncontrolled creation of thrombin and plasmin > intravascular fibrin > consumption of all hemostasis products > activation of lysis
35
conditions assoc. with DIC
excessive TF release - sepsis - trauma - CA - obstetrical - vascular - immune - toxins/drugs
36
test for DIC
none | - clinical + abnormal hemo screening
37
treat of DIC
treat underlying cause
38
what is vit K funciton
facilitates carbolxylation of 6 factors (10,9,7,2,C,S)
39
5 causes of vit K def.
1. warfarin 2. poor intake 3. ABs 4. biliary obstruction 5. hemmoragic disease of newborn
40
3 lab findings in vit K def.
1. long IRN 2. long PTT 3. low factos 10,9,7,2
41
what is DDx for long PTT but normal PT
1. single factor def. (inheritied) 2. VW disease 3. inhibirots - aquired - drug (hepain) -
42
DDx for long PT, but normal PTT
inherited or aquired factor 7 def.
43
DDx for long PTT and PT
1. inherited def. of common pathway 2. inherited abnormal fobrinogen 3. aquired - inhibitors - vit K def - too much warfarin - servere liver - DIC

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