Vasculopathies

Question 1

Disseminated Intravascular coagulation is typically initiated through:

Answer 1

Excessive Tissue Factor in the plasma

Question 2

The morbidity associated with DIC is

Answer 2

Hemorrhage due to lack of functional clotting factor

Question 3

Acute DIC can be treated as a stand-alone diagnosis

Answer 3

No, DIC is associated with an underlying condition that required treatment

Question 4

The most common trigger for DIC is

Answer 4

Infection

Question 5

(T or F) Low-grade DIC is often symptomatic

Answer 5

F

Question 6

(T or F) Low-grade DIC is enhanced by the coexistence of liver disease, and is typically an incidental finding

Answer 6

True

Question 7

Laboratory findings in acute DIC include

Answer 7

Prolonged PT, prolonged aPTT

Question 8

Which of the following tests is the most helpful in the workup of chronic DIC?

Answer 8

Presence of fibrin degradation products (FDP)

Question 9

What type of cells might you see in a peripheral blood smear in DIC?

Answer 9

Schistocytes

Question 10

What is the role of ADAMTS13?

Answer 10

Cleaves von Willebrand factor and prevents overactivity of platelet aggregation

Question 11

Deficiency of ADAMST13 is associated with which disease?

Answer 11

Thrombotic thrombocytopenic purpura (TTP)

Question 12

Primary idiopathic TTP has a higher incidence in which population

Answer 12

African Americans
Obese
Females

Question 13

The prognosis associated with TTP has been greatly improved with the utilization of

Answer 13

Plasmapheresis

Question 14

Features included in the “classic pentad” of TTP

Answer 14

Neurologic findings
Thrombocytopenia 
Fever
Anemia
Renal failure

Question 15

What are findings on a peripheral smear that are highly suspicious for TTP?

Answer 15

Absence of platelets

Schistocytes

Question 15

What are findings on a peripheral smear that are highly suspicious for TTP?

Answer 15

Absence of platelets

Schistocytes

Question 16

Thrombocytopenia in ITP is due to

Answer 16

Removal of platelets by the spleen

Question 17

(T or F) ITP can present at any age and presents differently in children and adults

Answer 17

True

Question 18

The correct order according to size of bruising from largest to smallest is

Answer 18

Hematoma > purpura > petechiae

Question 19

ITP is diagnosed by

Answer 19

It is a diagnosis of exclusion

Question 20

What percentage of children have ITP that self-resolves

Answer 20

80%

Question 21

Which condition is the most common cause of acute renal injury in children?
TTP 
DIC
HUS
ITP

Answer 21

HUS - Hemolytic Uremic Syndrome

Question 22

HUS differs from ITP in that

Answer 22

The platelet destruction is toxin mediated in HUS rather that autoimmune mediated

Question 23

Which conditions are considered thrombotic microangiopathies (TMAs)?

Answer 23

HUS and TTP

Question 24

(T or F) Atypical HUS is more likely than typical HUS to result in end-stage renal disease

Answer 24

True

Question 25

E. coli is generally transmitted to humans through

Answer 25

Ingestion of contaminated foods

Question 26

Why are kidneys particularly susceptible to injury from HUS

Answer 26

Gb3 , the binding site for shiga toxin, exists in high quantities on renal cells

Question 27

Which of the following is NOT true concerning the differences between HUS and TTP?

a. HUS does not typically present with the widespread symptoms associated with TTP
b. TTP etiology involves the clotting cascade, HUS does not
c. ADAMTS13 deficiency is seen in TTP but will be sufficient in HUS
d. HUS typically involves greater damage to the endothelium

Answer 27

b. TTP etiology involves the clotting cascade, HUS does not

Question 28

50-70% of patients with diarrhea associated HUS require ______ for treatment

Answer 28

Dialysis therapy

Question 29

Describe the pathophysiology of atypical HUS

Answer 29

Autoimmune or genetic mutation reducing complement cascade inhibitors