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Skin, Lymph, Blood > Dr. Pence - Detection, Dx, Management Of Bleeding And Clotting Disorders > Flashcards

Dr. Pence - Detection, Dx, Management Of Bleeding And Clotting Disorders Flashcards

1
Q

Key signs that someone has a bleeding disorder

A
  1. Heavy menstruation
  2. Epistaxis (nose bleeding)
  3. Gingival bleeding
  4. 5 or more visible bruises larger then 1cm
  5. Spontaneous Hemarthrosis **
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2
Q

If I see a patient with Spontaneous Hemarthrosis

A

= bleeding disorder Hemophilia type A (Factor 8 ) or Hemophilia B (Factor 9 deficiency)

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3
Q

Primary Hemostasis deficit

A

Platelet disorder, something is wrong with the amount or quality of platelets

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4
Q

Secondary Hemostatic Deficit

A

Coagulation factor deficiency or inhibitor (autoimmune)

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5
Q

Tertiary Hemostatic deficiency

A

CT or vascular disorder (autoimmune)

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6
Q

PT and PTT

A

PT : extrinsic coagulation pathway bleeding time

PTT : intrinsic coagulation pathway bleeding time

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7
Q 
CASE 1 : 13yo F fatigue, easy bruising, 1-2 times a month nose bleeds like her dad, scrapes take time to heal, heavy menstrual bleeding 
Hgb: 12.7 (14-18)
Plt : 270,000 (140,000-450,000)
PTT : 64sec (32-45)
Increased bleeding time 
PT : 13sec (10-14)
A

VON WILLEBRAND FACTOR DEFICIENCY
vWF Ag : decreased
vWF activity : decreased

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8
Q

How does Von Willebrand factor work

A
  1. It attaches to the subendothelial collagen and uncoils
  2. Then the platelet GP1a attaches to it
  3. The platelet gets closer to subendothelium and its integrin aB attaches to it : activated
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9
Q

How to test for vWB Deficiency

A
  1. Test for Ag : low means not a lot of factors there
  2. Test cofactor : see if the vWB factor functions right
  3. Test Factor 8 : coiled vWB protects F8, so if vWB is low so is F8 (Ag/activity)
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10
Q

How to TX minor bleeding

And the side effects

A
  1. DDAVP : Desmopressin =
    - allow release of vWB from endothelial cells and platelets
    - tachyphlaxis : less effective over time, so temporary tx
    - Side Effects: flushing, Hypotension, hyponatremia(too much ADH)*
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11
Q

How to TX major bleeding

And the side effects

A

Plasma derived : donor or recombinant vWF- cultured in lab

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12
Q

vWB inheritance

A

Autosomal Dominant

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13
Q 
CASE 2 : 
6yo males with oral bleeding after dental procedure 
ED visit for painful enlarged knee
Normal BP and pulse
Many bruises on abd and extremities (no abuse)
PT : 13sec (10-14)
PTT : 89sec (32-45)
Factor 8 : 3% normal 
Bleeding time : normal
A

Hemophilia A

Secondary Hemostasis problem

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14
Q

Intrinsic pathway factors and time

A

12,11, 9, 8

aPartial Thromboplastin Time

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15
Q

Extrinsic pathway factors and time

A

Tissue Factor —> F7

Prothrombin Time

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16
Q

Common pathway factors and time

A

F 10, 5 ,Ca+3, Lipids, THROMBIN (3), Fibrinogen (1), Prothrombin (2)

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17
Q

Prolonged PT and PTT can mean what

A

Anticoagulants, DIC, liver diseases, VIT K deficiency, massive transfusion , factor 10,5,2 deficiency

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18
Q

How to confirm hemophilia A

A

3% factor 8

98% factor 9

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19
Q

Hemophilia A can be confused with and how to tell them apart

A

vWF since you can see normal vWB antigen and activity and very low Factor 8 : INCREASED BLEEDING TIME

Hemophilia A : normal bleeding time

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20
Q

How is Hemophilia A passed on

A

X- linked recessive

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21
Q

Hemophilia TX

A

Increase Factors for Hemostasis (becomes mild bleeding)
= recombinant F8*
= plasma transfusion : not used anymore due to HIV pandemic and infections
= Emicizumab : works like F8 and binds F9a and F10, effective, pricy
= Gene therapy in future (insert virus with F8 DNA)

22
Q 
CASE 3 : 32 yo F heavy menstrual bleeding 
Bled 10 days postpartum heavily
Recurrent bleeding 6 weeks later
Nosebleeds, fatigue, oral bleeding (all after birth) 
Period cycle : normal and moderate
Mom—> RA, Dad —> HTN, sister—> SLE
PE : conjunctival pallor, bruises on legs, vaginal bleeding
Hgb : 10.8 (14-18), mild anemia
Hct : 32.9% (37-54)
PT : 12.5 (10-14)
PTT : 42 (21-23)long
Mixing PTT : did not correct
Normal bleeding time
A

+ F8 inhibitor

= acquired Hemophilia A (Acquired Factor 8 Inhibitor)

23
Q

Mixing PTT test shows what **

A

You add prolonged PTT + normal PTT plasma from someone and see if the time corrects itself or not

  1. It corrects : factor deficiency
  2. IT does not correct: plasma problem something in it inhibiting the factors
24
Q

acquired Hemophilia A (Acquired Factor 8 Inhibitor) TX

A
  1. If they are to stable : give fluids
  2. Low titer inhibitor : give F8
  3. High titer inhibitor: F8 bypass, by giving them F7 + immunosuppressant
25
Q

What are the risk factors for acquired Hemophilia A (Acquired Factor 8 Inhibitor)

A
  1. RA, SLE, other autoimmune diseases
  2. Postpartum
  3. Older adults
  4. Malignancy
  5. People with congenital hemophilia A
26
Q

Drug induced inhibition of factors EX

Most common autoimmune factor problems

A

Heparin
Warfarin

Factor 8 and factor 5

27
Q

Proximal DVT is in what vessels

Distal veins

A

Femoral, popliteal, iliac
Fibulae, posterior tibial, anterior tibial
VEINS

28
Q

How to make sure its not a muscle strain

A
  1. Measure circumstance of both legs

2. Screen for risk factors

29
Q

Pulmonary embolism can cause

A

Acute hypoxia + right heart failure

30
Q

Criteria to DX a VTE

A 
Use Wells and Geneva criteria
Risk factors : 
- obesity
- recent hospitalization 
- malignancy 
- prior PE or DVT
- over 65yo
31
Q

How do I go about diagnosing a VTE

A
  1. Risk factors present : imaging test

2. No risk factors present : test D-dimer and if high then do imaging

32
Q

Imaging for VTE

A
  1. US for DVT

2. CT angiogram for PE

33
Q

D-Dimer test (high - predictive value)

A

A lot of fibrinogen degradation = high D-dimer

= a lot of clotting happening

34
Q

How to see a blood clot on the US

A

You cant compress an A on the US, however you can compress a vein
When there is a blood clot the Vein does not compress

35
Q

How to see PE on the CT

A

Normally all arteries and vessels are white

However a clot will show up as darker gray area in the vessels

36
Q

PE imaging if patient cant tolerate CT

A

Do V/Pq test : ventilation / perfusion test :NOT for COPD
Not diagnostic only how likely it could be a clot

V/Q mismatch is a high probability

37
Q

CASE 4 : 36yo F abd pain, thrown up twice in ED, started 4 hours ago, worsening,
- had spontaneous miscarriage 12 years ago
Mx: MVI, oral contraceptives
MOM : died from stroke at 45yo no smoking and was healthy
- occult blood in stool
Elevated WBC, N, and bands = left shift
SI obstruction

A

Clot in the mesenteric vein : in surgery saw she has that
Protein C deficiency
Thrombophilia

38
Q

What ones Anti-phospholipid Abs do

A

Interferes with Protein C, fibrinolysis, and can cause spontaneous miscarriage, increased platelet activation, SLE

39
Q

Protein C and Protein S

A

They block F8 and F5
Synthesized in the liver
Deficiency = thrombophilia

40
Q

White thrombus
Where
What

A 
Arterial
Platelet rich + fibrin streaks
Usually in high shear flow or stress
Atherosclerosis**
- causes MI or stroke
41
Q

Red thrombus

A 
Venous blood
High in RBC
Due to stasis of blood (low shear flow)
Lower extremities usually
- causes DVT, PE
42
Q

Budd-chiari syndrome

A

Clot in the liver portal vein
Such clots and othe deep visceral vein clots (like the mesentery one) is a high sign someone has a congenital hypercoagulatory disease

43
Q

Peridoxical Embolism
How
And what
And DX

A

Stroke from DVT
- if you have an open foramen ovale (PFO)
DX : with TEE = transesophageal echocardiography + bubble study

44
Q

If a patient has a stroke at the age of under 55yo what should you look like

A

Patent Foramen Ovale

45
Q

When to test a patient for hypercoagable state

A
  • not usually esp if patient has any audited risk factors like flying after a surgery
  • yes f patents is younger then 40yo, FH
46
Q

How to TX hypercoagability state patients

4 ways

A
  1. Heparin : inhibits 2,9,10,11
  2. Factor 10a inhibitor : Rivaroxaban, Apixaban** GOLD STANDARD (no diet modification, works well)
  3. Thrombin inhibitor : Dabigatran
  4. Warfarin : inhibits VIT K dependent factors (2,7,9,10), (need dietary modifications, and frequent blood checkups)
47
Q

How long do you TX patients with hypercoagability states**

A
  1. 3-6 months - provoked
  2. Lifetime - 2 or more spontaneous VTEs, 1 unusual thrombosis (esp deep visceral veins), 1 thrombosis with congenital defect)
48
Q

Antiplatelet drugs

A

Aspirin , esp after coronary surgery

49
Q

IVC filters

What

A

Used for patients that should not be on anticoagulants (has had brain tumor surgery and at risk of brain bleeding, thrombocytopenia) + have high risk of blood clotting

50
Q

IVC risks

A

Can completely embolize itself, can break, can move

Skin, Lymph, Blood (9 decks)

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