Lecture 3c

Question 1

Define hemophilia A.

Answer 1

Congenital deficiency of coagulation F8

Question 2

Define hemophilia B.

Answer 2

Congenital deficiency of coagulation F9

Question 3

What is the common symptom of hemophilia?

Answer 3

Recurrent hemarthoses(joint bleeding)
Easy bruising/bleeding

Question 4

How does severe hemophilia present in infant males or early childhood?

Answer 4

Spontaneous bleeding into joints, soft tissue

Question 5

Why is an associated negative development that could occur with hemophilia?

Answer 5

Develop inhibitors to the associated clotting factor
30% of pts in hemophilia A
<5% in hemophilia B

Question 6

What is the normal range for F8?

Answer 6

50-150% (varies with age)

Question 7

How do you treat hemophilia?

Answer 7

Plasma-derived or recombinant factor concentrates
Can be treated with prophylaxis
Severe hemophilia can come up to 3x a week

Question 8

Can pts live a normal life with hemophilia?

Answer 8

Yes, if prophylactically treated/managed and educated

Question 9

What is the MC cause of death in hemophilia pts?

Answer 9

Transfusion-obtained HIV/AIDs
Hepatitis/cirrhosis

Question 10

What must hemophilic pts do to avoid complications?

Answer 10

Avoid contact sports
Monitor for S/S of bleeding
Home infusion technique
Managed by hematologist

Question 11

What is the MC inherited bleeding disorder?

Answer 11

Von willebrand disease

Question 12

How many types of von willebrand stages are there?

Answer 12

3
Type 1: quantitive(not enough)
Type 2: qualitative(dysfunctional)
Type 3: profound quantitive(near or total absence of VWF)

Question 13

What is F11 deficiency also referred to as?

Answer 13

Hemophilia C

Question 14

What does thrombocytopenia present as?

Answer 14

Mucous membrane related bleeding…
Epistaxis(nose bleed)
Gum bleeding
GI bleeds
Life-threatening cerebral hemorrhage

Question 15

How can thrombocytopenia occur?

Answer 15

Increased destruction
Decreased production

Question 16

What are examples that decrease production of platelets?

Answer 16

Congenital/Acquired bone marrow failure
Exposure to chemotherapy, irradiation
Marrow infiltration
Nutritional

Question 17

What are examples of destructive/consumption thrombocytopenia?

Question 18

How does splenomegaly/hypersplenism destroy platelets?

Answer 18

Aged platelets are normally destroyed by the spleen, but it is now unregulated

Question 19

What is the etiology of immune thrombocytopenia purpura?

Answer 19

Pts form auto-AB against Ag on the platelet surface (causing destruction)

Question 20

What lab do we order for suspicion of immune thrombocytopenia purpura?

Answer 20

CBC with peripheral blood smear

Question 21

Is there a cure for ITP?

Answer 21

No

Question 22

What are the treatments for ITP?

Answer 22

Corticosteroids(first line)
IVIG
Platelet transfusion(serious bleeds)
Splenectomy

Question 23

What drugs are the common cause of acquired/iatrogenic PLT defects?

Answer 23

Aspirin
Clopidogrel
NSAIDs

Question 24

What thrombotic microangiopathies (TMAs) are there?

Answer 24

Thrombotic thrombocytopenic purpura (TTP)
Hemolytic-uremic syndrome (HUS)

Question 25

How do you diagnose TTP?

Answer 25

CDC (should show elevated WBC) Hg 8-9g/dL PLT 20-50k Peripheral smear (presence of schistocytes) Coagulation studies, PT, aPTT are normal D-dimer (normal-elevated) Fibrinogen (high) LDH (high) Direct Coombs test negative Hyperbilirubinemia

Question 26

What is the most common cause of acute renal failure in children?

Answer 26

Hemolytic-uremic syndrome(HUS)

Question 27

How does HUS present?

Answer 27

Prodromal gastroenteritis Prodrome of fever, bloody diarrhea for 2-7 days before onset of renal failure Irritability, lethargy Seizures Acute renal failure Anuria HTN Edema/volume overload Pallor

Question 28

What bacteria is the most common cause of HUS in NA and Western Europe?

Answer 28

E. coli serotype 0157:H7

Question 29

What disorders are associated with DIC?

Answer 29

Sepsis Cancer, trauma, burns, or pregnancy-assocated morbidity Aortic aneurysm or cavernous hemangiomas Snake bites