Coagulation And Bleeding Disorders

Front 1

Components of coagulation system

Back 1

(1) Vasculature

(2) Platelets

(3) Clotting pathway

Front 2

Role of platelets in hemostasis

Back 2

(1) Platelet adhesions to subendothelial connective tissue

(2) Release of granules (ADP, Thromboxane)

(3) Platelet Aggregation

(4) Stabilization of hemostatic plug

(5) Stimulation of limiting reactions

Front 3

Clotting cascade

Back 3

Front 4

Thrombin sensitive factors

Back 4

I, V, VIII, XIII

Front 5

Vitamin K sensitive factors

Back 5

II, VII, IX, X

Front 6

Instrinsic Clotting Factors

Back 6

XII, XI, IX, VIII

Front 7

Extrinsic Clotting Factors

Back 7

VII

Front 8

Common Pathway Clotting Factors

Back 8

X, V, II (Thrombin), I (Fibrinogen)

Front 9

Activity of Protein C & S

Back 9

Inhibit cofactors Va and VIIIa

Front 10

Action of AntiThrombin

Back 10

inhibits the serine proteases (factors II, IX, X, XI, and XII);

its anticoagulant action is dramatically enhanced by heparin

Front 11

Control of excessive thrombis

Back 11

(1) Removal/dilution of activated clotting factors through blood flow
(2) Modulation of platelet activity by endothelium-generated nitric oxide and prostacyclin

(3) Removal of activated coagulation components by the reticuloendothelial system

(4) Regulation of the clotting cascade by antithrombin III, protein C, protein S, and tissue factor pathway inhibitor

(5) Activation of the fibrinolytic system (plasmin degredation)

Front 12

Differential diagnosis of vascular disorders

Back 12

Inherited
Disorders of connective tissue
Pseudoxanthoma elasticum
Ehlers-Danlos syndrome
Osteogenesis imperfecta
Disorders of blood vessels
Hemorrhagic telangiectasia

Acquired
Scurvy (vitamin C deficiency)
Simple or senile purpura
Purpura secondary to steroid use
Vascular damage
Infection (meningococcemia)
Hemolytic-uremic syndrome
Hypoxemia
Thrombotic thrombocytopenic purpura
Snakebite
Dysproteinemic purpura

Front 13

Differential Diagnosis of Platelet Disorders

Back 13

Thrombocytopenia

Decreased production
Decreased megakaryocytes secondary to drugs, toxins, or infection
Normal megakaryocytes with megaloblastic hematopoiesis or hereditary origin
Platelet pooling and splenic sequestration

Increased destruction
Immunologic
Related to collagen vascular disease, lymphoma, leukemia
Drug related
Infection
Post-transfusion
Idiopathic (autoimmune) thrombocytopenic purpura

Mechanical
Disseminated intravascular coagulation
Thrombotic thrombocytopenic purpura
Hemolytic-uremic syndrome
Vasculitis
Dilutional secondary to massive blood transfusion

Thrombocytopathy
Adhesion defects such as von Willebrand’s disease
Release defects: acquired and drug related
Aggregation defects such as in thrombasthenia

Thrombocytosis
Autonomous (primary thrombocythemia)
Reactive (secondary thrombocythemia)
Iron deficiency
Infection or inflammation
Trauma
Nonhematologic malignant disease
Postsplenectomy
Rebound from alcohol, cytotoxic drug therapy, folate or vitamin B12 deficiency

Front 14

Platelet lifespan

Back 14

7-10 Days

Front 15

4T's Score for HIT

Back 15

Front 16

Treatment principles for HIT

Back 16

1. Discontinue and avoid all heparin.
2. Give a nonheparin alternative
anticoagulant.
3. Postpone warfarin pending substantial platelet count recovery (give vitamin K if warfarin has already been started).
4. Test for HIT antibodies.
5. Investigate for lower-limb deep-vein thrombosis.
6. Avoid prophylactic platelet transfusions.

Front 17

MOA of HIT

Back 17

IgG antibodies that bind to platelet factor 4/heparin complex activating platelets and triggering a pro-coagulant response

Front 18

Algorithim using 4 t's score

Back 18

0-3: Consider alternate Dx

4 or greater: D/C Heparin and W/U

Front 19

Rx of significant bleeding in patient with ITP

Back 19

Platelets

IVIG

Cortiosteroids

Front 20

Classic pentad of TTP

Back 20

(1) fever

(2) hemolytic anemia

(3) thrombocytopenia

(4) renal impairment

(5) neurologic manifestations

Front 21

Causes of TTP

Back 21

Idiopathic—most cases
Infection
- HIV
Pregnancy
Malignancy
Bone marrow transplantation
Drug therapy
- Quinine
- Oral contraceptive pills
- Clopidogrel
- Ticlopidine
- Trimethoprim
- Pegylated interferon
- Simvastatin
Chemotherapy
Pancreatitis
Autoimmune
- Systemic lupus erythematosus
=- Antiphospholipid syndrome

Front 22

Pathophysiology of TTP

Back 22

MAHA + Thrombocytopenia without alternate explanation

Deficiency of ADAMTS-13, VWF cleaving protein, leads to platlet aggregation and occlusion of microvasculature

Front 23

TTP Management

Back 23

Plasma exchange

Corticosteroids

Rituximab in Consultation with hematology

Platelet Transfusion

Front 24

HUS

Back 24

MAHA

Thrombocytopenia

Bloody Diarrhea

Prominent Renal Failure

More common in children

Front 25

Medications implicated in ITP

Back 25

Acetaminophen
Acetazolamide
Allopurinol
Amiodarone
Ampicillin
Aspirin
Carbamazepine
Cephalexin
Chlorothiazide
Chlorpheniramine
Cimetidine
Diazepam
Digoxin
Diphenylhydantoin
Furosemide
Gentamicin
Heparin
Lidocaine
Methicillin
Methyldopa
Minoxidil
Morphine
Penicillin
Phenylbutazone
Procainamide
Quinidine
Quinine (tonic water)
Ranitidine
Rifampin
Spironolactone
Tricyclic antidepressants
Trimethoprim-Sulfamethoxazole
Valproic acid
Vancomycin

Front 26

DIC

Back 26

DIC is an acquired, systemic process of overstimulation of the coagulation pathway resulting in thrombosis, followed by consumption of platelets and coagulation factors, and ending in hemorrhage

Front 27

When to use heparin in DIC

Back 27

If evidence of thromboembolic disease, retained products of conception, or purpura fulminans (gangrene of digits and extremities) and requires a normal antithrombin level before initiation

Front 28

Effect of ASA on platelets

Back 28

Blocks enzyme cyclooxygenase, which participates in thromboxane A2 formation

Thereby decreases platelet aggregation

Front 29

Hemophilia A

Back 29

Deficient Factor VIII Activity

X linked recessive

Front 30

Severity of Hemophillia A by level of factor activity

Back 30

<1% severe (Frequent atraumatic bleeds)

1-5% Moderate (Bleed after minor trauma)

5-10% Mild (Bleed after significant trauma)

Front 31

How much will unit/kg of factor VIII:C raise the circulating factory VIII level

Back 31

2%

Front 32

Recommended Factor VIII therapy for specific problems in hemophilia from Rosen's

Back 32

Front 33

Monitoring response to therapy in Hemophilia

Back 33

Clinical Improvement

PTT

Factor VII coagulant activity

Front 34

Dosage guidelines for Factor VIII

Back 34

Front 35

Hemophilia B (Christmas disease)

Back 35

Deficient Factor IX Activity

Treatment is similar to Hemophilia A

Front 36

Laboratory findings in keeping with DIC

Back 36

Front 37

MOA of clopidogrel

Back 37

Inhibit ADP induced platelet aggregation

Front 38

Types of VWD

Back 38

Front 39

Lab Abnormalities in Congenital Bleeding Disorders

Back 39

Front 40

Simplified Factor Level recommendation for Hemophilia from EMNA

Back 40

Oral mucosa >30
Epistaxis >30
Joint or muscle >50
GI >50
GU >50
CNS >100
Trauma or surgery >100

Front 41

Treatment of Von Willebrand

Back 41

Type 1 - DDAVP
Type 2/3 - VWF Replacement

Front 42

Treatment in patients with Factor inhibitors (antibodies)

Back 42

aPCC (octoplex)

rFVIIa

Front 43

Hemophiliac patients who are candidates for prophylactic admissions

Back 43

Deep lacerations

Soft tissue in areas compromised by expanding hematomas

- Eye

- Airway

- Spinal Column

- Head trauma