Bleeding Disorders

Question 1

Measures effectiveness of the Intrinsic Pathway.

Answer 1

Partial Thromboplastin Time (PTT)
(PITT = Intrinsic)

Question 2

Measures effectives of the Extrinsic Pathway.

Answer 2

Prothrombin Time (PT)
(PET = Extrinsic)

Question 3

X-linked recessive disorder that affects the clotting cascade due to a lack of Factor VIII (8). Occurs predominantly in males.

Answer 3

Hemophilia A

Question 4

X-linked recessive disorders that affects the clotting case due to a lack of Factor IX (9). Occurs almost exclusively in males.

Answer 4

Hemophilia B

Question 5

What will a CBC and Coagulation study shown in patients with Hemophilia?

Answer 5

Prolonged PTT
Normal bleeding time

Question 6

What medication can be used to increase Factor III (8) and von Willebrand Factor in patients with Hemophilia A?

Answer 6

Desmopressin (DDAVP)

Question 7

What is the first line therapy for Hemophilia A

Answer 7

Factor VIII (8) Infusion

Question 8

What is the first line therapy for Hemophilia B

Answer 8

Factor IX (9) Infusion

Question 9

What type of bleeding is seen in patients with Hemophilia

Answer 9

Deep Tissue Bleeding
Soft Tissue Bleeding

Question 10

Most common inherited cause of hypercoagulability (thrombophilia) especially in Whites.

Answer 10

Factor V Leiden Mutation
(Activated Protein C Resistance)

Question 11

What is the pathophysiology of Factor V Leiden Mutation?

Answer 11

Mutated activated Factor V is resistant to normal breakdown by Protein C

Question 12

What are the signs and symptoms of Factor V Leiden Mutation?

Answer 12

Venous Thromboembolism
DVT
Pulmonary Embolism
Thrombosis in Unusual Veins

Question 13

How do you diagnose Factor V Leiden Mutation?

Answer 13

Activated Protein C Resistance Assay
(confirm with DNA testing)

Question 14

How do you treat a patient for Factor V Leiden Mutation?

Answer 14

Anticoagulation Medications
(Oral or Warfarin)

Question 15

Inherited Autosomal Recessive disorder most prevalent among individuals of Ashkenazi Jewish descent. Mild bleeding is most common, and diagnosis is often made after unexpected, excessive bleeding following surgery or trauma

Answer 15

Hemophilia C (Factor XI)

Question 16

What may not be prolonged in Hemophilia C (Factor XI) that is prolonged with Hemophilia A and B?

Answer 16

Partial Thromboplastin Time (PTT)

Question 17

What is the mainstay of treatment for patients with Hemophilia C (Factor XI)

Answer 17

Factor XI Replacement
Fresh Frozen Plasma if it is not available
Adjunctive Aminocaproic Acid or Tranexamic Acid

Question 18

Most commonly inherited bleeding disorder (autosomal dominant) with ineffective platelet adhesion due to a specific deficient or defective factor.

Answer 18

von Willebrand Disease

Question 19

What types of bleeding problems are especially common in patients with von Willebrand Disease?

Answer 19

Gynecologic

Question 20

What factor may be slightly low in a patient with von Willebrand Disease?

Answer 20

Factor VIII (8)

Question 21

What is the criterion standard for the diagnosis of von Willebrand Disease?

Answer 21

Ristocetin-Induced Platelet Aggregation

Question 22

What is the primary treatment medication for a patient with Mild to Moderate von Willebrand Disease?

Answer 22

Desmopressin

Question 23

What is the primary treatment of a patient with severe von Willebrand Disease or a patient who lacks von Willebrand Factor entirely?

Answer 23

Infusion of von Willebrand Factor-containing product

Question 24

An acquired Thrombocytopenia that typically occurs within the first 5 - 10 days of initiation of a specific anticoagulant

Answer 24

Heparin Induced Thrombocytopenia

Question 25

Heparin Induced Thrombocytopenia is caused by the formation of autoantibodies that are attacking what targets?

Answer 25

Hapten Complex of Heparin + Platelet Factor 4

Question 26

What happens to the platelets during Heparin Induced Thrombocytopenia?

Answer 26

Platelets drop by at least 50% in 5 - 10 days after Heparin treatment

Question 27

What is the Gold Standard test for the diagnosis of Heparin Induced Thrombocytopenia?

Answer 27

Serotonin Release Assay

Question 28

What are the 4 Ts of Predicting Heparin Induced Thrombocytopenia?

Answer 28

Thrombocytopenia
Timing of Platelet Drop
Thrombosis
OTher causes Absent

Question 29

A 4Ts score of 6 - 8 would indicated what with HIT?

Answer 29

High Probability

Question 30

A 4Ts score of 4 - 5 would indicate what with HIT?

Answer 30

Intermediate Probability

Question 31

A 4Ts score of 0 - 3 would indicate what with HIT?

Answer 31

Low Probability

Question 32

How do you treat Heparin Induced Thrombocytopenia?

Answer 32

Stop Heparin + Start Agatroban
Order diagnostic test
Doppler all 4 extremities for thrombosis

Question 33

Consumptive coagulopathy due to uncontrolled fibrin production leading to widespread micro-thrombi and clotting factors as well as platelets are consumed.

Answer 33

Disseminated Intravascular Coagulation (DIC)

Question 34

What infections are common causes of Disseminated Intravascular Coagulation?

Answer 34

Bacterial Sepsis
Rocky Mountain Spotted Fever

Question 35

In most cases of Disseminated Intravascular Coagulation, what gains entry into the blood?

Answer 35

Pro Coagulant Tissue Factor
(lipoprotein)

Question 36

What are the signs and symptoms of DIC?

Answer 36

Diffuse Bleeding
Thrombosis
Gangrene
Multi-organ Dysfunction

Question 37

What lab value will be markedly elevated in a patient with DIC?

Answer 37

D-Dimer

Question 38

What would a peripheral smear show in a patient with DIC?

Answer 38

Schistocytes
Fragmented Red Blood Cells

Question 39

How will DIC affect:
PT
PTT
Bleeding Time
Platelet Count

Answer 39

PT = Increased
PTT = Increased
Bleeding Time = Increased
Platelet Count = Decreased

Question 40

What is the treatment for DIC?

Answer 40

Supportive (Treat the Underlying cause)
Platelet Transfusion
Fresh Frozen Plasma
Cryoprecipitate to replace Fibrinogen