Dr. Pence - Detection, Dx, Management Of Bleeding And Clotting Disorders Flashcards
Key signs that someone has a bleeding disorder
- Heavy menstruation
- Epistaxis (nose bleeding)
- Gingival bleeding
- 5 or more visible bruises larger then 1cm
- Spontaneous Hemarthrosis **
If I see a patient with Spontaneous Hemarthrosis
= bleeding disorder Hemophilia type A (Factor 8 ) or Hemophilia B (Factor 9 deficiency)
Primary Hemostasis deficit
Platelet disorder, something is wrong with the amount or quality of platelets
Secondary Hemostatic Deficit
Coagulation factor deficiency or inhibitor (autoimmune)
Tertiary Hemostatic deficiency
CT or vascular disorder (autoimmune)
PT and PTT
PT : extrinsic coagulation pathway bleeding time
PTT : intrinsic coagulation pathway bleeding time
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)
VON WILLEBRAND FACTOR DEFICIENCY
vWF Ag : decreased
vWF activity : decreased
How does Von Willebrand factor work
- It attaches to the subendothelial collagen and uncoils
- Then the platelet GP1a attaches to it
- The platelet gets closer to subendothelium and its integrin aB attaches to it : activated
How to test for vWB Deficiency
- Test for Ag : low means not a lot of factors there
- Test cofactor : see if the vWB factor functions right
- Test Factor 8 : coiled vWB protects F8, so if vWB is low so is F8 (Ag/activity)
How to TX minor bleeding
And the side effects
- 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)*
How to TX major bleeding
And the side effects
Plasma derived : donor or recombinant vWF- cultured in lab
vWB inheritance
Autosomal Dominant
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
Hemophilia A
Secondary Hemostasis problem
Intrinsic pathway factors and time
12,11, 9, 8
aPartial Thromboplastin Time
Extrinsic pathway factors and time
Tissue Factor —> F7
Prothrombin Time
Common pathway factors and time
F 10, 5 ,Ca+3, Lipids, THROMBIN (3), Fibrinogen (1), Prothrombin (2)
Prolonged PT and PTT can mean what
Anticoagulants, DIC, liver diseases, VIT K deficiency, massive transfusion , factor 10,5,2 deficiency
How to confirm hemophilia A
3% factor 8
98% factor 9
Hemophilia A can be confused with and how to tell them apart
vWF since you can see normal vWB antigen and activity and very low Factor 8 : INCREASED BLEEDING TIME
Hemophilia A : normal bleeding time
How is Hemophilia A passed on
X- linked recessive
Hemophilia TX
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)
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
+ F8 inhibitor
= acquired Hemophilia A (Acquired Factor 8 Inhibitor)
Mixing PTT test shows what **
You add prolonged PTT + normal PTT plasma from someone and see if the time corrects itself or not
- It corrects : factor deficiency
- IT does not correct: plasma problem something in it inhibiting the factors
acquired Hemophilia A (Acquired Factor 8 Inhibitor) TX
- If they are to stable : give fluids
- Low titer inhibitor : give F8
- High titer inhibitor: F8 bypass, by giving them F7 + immunosuppressant
What are the risk factors for acquired Hemophilia A (Acquired Factor 8 Inhibitor)
- RA, SLE, other autoimmune diseases
- Postpartum
- Older adults
- Malignancy
- People with congenital hemophilia A
Drug induced inhibition of factors EX
Most common autoimmune factor problems
Heparin
Warfarin
Factor 8 and factor 5
Proximal DVT is in what vessels
Distal veins
Femoral, popliteal, iliac
Fibulae, posterior tibial, anterior tibial
VEINS
How to make sure its not a muscle strain
- Measure circumstance of both legs
2. Screen for risk factors
Pulmonary embolism can cause
Acute hypoxia + right heart failure
Criteria to DX a VTE
Use Wells and Geneva criteria Risk factors : - obesity - recent hospitalization - malignancy - prior PE or DVT - over 65yo
How do I go about diagnosing a VTE
- Risk factors present : imaging test
2. No risk factors present : test D-dimer and if high then do imaging
Imaging for VTE
- US for DVT
2. CT angiogram for PE
D-Dimer test (high - predictive value)
A lot of fibrinogen degradation = high D-dimer
= a lot of clotting happening
How to see a blood clot on the US
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
How to see PE on the CT
Normally all arteries and vessels are white
However a clot will show up as darker gray area in the vessels
PE imaging if patient cant tolerate CT
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
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
Clot in the mesenteric vein : in surgery saw she has that
Protein C deficiency
Thrombophilia
What ones Anti-phospholipid Abs do
Interferes with Protein C, fibrinolysis, and can cause spontaneous miscarriage, increased platelet activation, SLE
Protein C and Protein S
They block F8 and F5
Synthesized in the liver
Deficiency = thrombophilia
White thrombus
Where
What
Arterial Platelet rich + fibrin streaks Usually in high shear flow or stress Atherosclerosis** - causes MI or stroke
Red thrombus
Venous blood High in RBC Due to stasis of blood (low shear flow) Lower extremities usually - causes DVT, PE
Budd-chiari syndrome
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
Peridoxical Embolism
How
And what
And DX
Stroke from DVT
- if you have an open foramen ovale (PFO)
DX : with TEE = transesophageal echocardiography + bubble study
If a patient has a stroke at the age of under 55yo what should you look like
Patent Foramen Ovale
When to test a patient for hypercoagable state
- not usually esp if patient has any audited risk factors like flying after a surgery
- yes f patents is younger then 40yo, FH
How to TX hypercoagability state patients
4 ways
- Heparin : inhibits 2,9,10,11
- Factor 10a inhibitor : Rivaroxaban, Apixaban** GOLD STANDARD (no diet modification, works well)
- Thrombin inhibitor : Dabigatran
- Warfarin : inhibits VIT K dependent factors (2,7,9,10), (need dietary modifications, and frequent blood checkups)
How long do you TX patients with hypercoagability states**
- 3-6 months - provoked
- Lifetime - 2 or more spontaneous VTEs, 1 unusual thrombosis (esp deep visceral veins), 1 thrombosis with congenital defect)
Antiplatelet drugs
Aspirin , esp after coronary surgery
IVC filters
What
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
IVC risks
Can completely embolize itself, can break, can move
