Non-malignant Hematology

Question 1

What mutations can occur in tMDS from alkylating agents?

Answer 1

Monosomy 5 or 7

Question 2

What mutations can occur in tMDS from topoisomerase inhibitors?

Answer 2

translocation 11q23

Inversion chromosome 3

Question 3

What is one of the most common mutation in MDS with ringed sideroblasts?

Answer 3

SF3B1 - spliceosome

Question 4

DNMT3A mutation

Answer 4

Present in MDS 10-15% of the time. Poorer prognosis. No specific disease phenotype.

Question 5

What are side effects of deferasirox?

Answer 5

1. GI upset
2. Maculopapular rash
3. Decreased GFR
4. Rare hepatic injury / GIB

Question 6

What clinical factor predicts for response to TSAs in MDS?

Answer 6

Serum thrombopoeitin < 500

Question 7

Which form of vWD has a qualitative defect?

Answer 7

Type 2 vWD

Question 8

Which form of vWD has a proportionate decrease in Ristocetin cofactor assay along with vWF antigen?

Answer 8

Type 1 vWD

Question 9

Which form of vWD has a disproportionate decrease in Ristocetin cofactor assay along with vWF antigen?

Answer 9

Type 2 vWD

Question 10

Which form of vWD has nearly absent vWF antigen and nearly undetectable ristocetin cofactor assay?

Answer 10

Type 3 vWD

Question 11

Which platelet disorder will have normal results on platelet function assay EXCEPT for with vWD?

Answer 11

Bernard-Soulier

Question 12

Which platelet disorder will ONLY show normal results with ristocetin but abnormal with all other reagents?

Answer 12

Glanzmann thrombasthenia

Question 13

Which two coagulation factors lack concentrates?

Answer 13

Factor V

Factor XI

Question 14

What factor decreases in pregnancy? Which increases?

Answer 14

1. Protein C decreases

2. Plasminogen activator inhibitor increases (accounts for increased risk of VTE)