Hematology

Question 1

Reversal agent for dabigatran

Answer 1

idarucizumab (Praxbind)

Question 2

Reversal agent for apixaban/rivaroxaban

Answer 2

andexanet alfa (Andexxa)

Question 3

Indications for rFVIIa

Answer 3

patients with congenital hemophilia A or B with inhibitors, patients with acquired hemophilia, congenital factor VII deficiency, Glanzmann thrombasthenia w/platelet refractoriness

Question 4

Condition with thrombocytopenia and granulocyte dohle bodies

Answer 4

May-Hegglin anomaly (MYH9 gene)

Question 5

Prophylaxis needed with eculizumab

Answer 5

meningococcal (antibiotics or vaccine)

Question 6

What is passenger lymphocyte syndrome?

Answer 6

Alloimmune hemolysis of host erythrocytes by antibodies produced by donor lymphocytes; occurs in solid organ transplant recipients

Question 7

What is caplacizumab

Answer 7

anti-vWF antibody; used for treatment of TTP in combo with immunosuppression and PLEX

Question 8

Which subtypes of vWD may be inherited in a recessive manner?

Answer 8

type 2N and type 3

Question 9

What is the defect in type 1 vWD?

Answer 9

Partial quantitative deficiency of vWF (usually low secretion or increased clearance)

Question 10

What is the defect in type 3 vWD

Answer 10

virtually complete deficiency of vWF

Question 11

What is the defect in type 2A vWD?

Answer 11

Defect in multimerization or increased cleavage of multimers by ADAMSTS13

Question 12

What is the defect in Type 2B vWD?

Answer 12

Increased affinity for platelet GP1b

Question 13

What is the defect in Type 2M vWD?

Answer 13

Decreased vWF mediated platelet adhesion

Question 14

What is the defect in Type 2N vWD?

Answer 14

Markedly decreased binding affinity for FVIII

Question 15

Lab findings in Type 1 vWD?

Answer 15

VWF:AG <30, VWF:RCo <30, Ratio RCO:Ag >0.6, normal or slightly decreased FVIII, Normal Multimers, Normal RIPA

Question 16

Lab findings in Type 2A vWD?

Answer 16

VWF:AG 30-200, VWF:RCo <30, Ratio RCo:Ag <0.6, normal or slightly decreased FVIII, loss of high molecular weight multimers, decreased RIPA

Question 17

Lab findings in Type 2B vWD?

Answer 17

VWF:AG 30-200, VWF:RCo <30, Ratio RCo:Ag <0.6, normal or slightly decreased FVIII, loss of high molecular weight multimers, increased RIPA

Question 18

Lab findings in Type 2M vWD?

Answer 18

VWF:AG 30-200, VWF:RCo <30, Ratio RCo:Ag <0.6, normal or slightly decreased FVIII, normal multimers, decreased RIPA

Question 19

Lab findings in Type 2N vWD?

Answer 19

VWF:AG 30-200, VWF:RCo 30-200, Ratio RCo:Ag >0.6, decreased FVIII, normal multimers, normal RIPA

Question 20

Lab findings in Type 3 vWD?

Answer 20

VWF:AG <5 VWF:RCo <5 Ratio RCo:Ag N/A, very decreased FVIII, absent multimers, absentRIPA

Question 21

What is platelet-type vWD?

Answer 21

rare disorder caused by gain of function mutation in GpIB that mimics vWD type 2B

Question 22

How is the diagnosis of platelet type vWD confirmed?

Answer 22

Enhanced binding of exogeneous VWF substrate to patient’s platelets in a ristocetin-induced platelet agglutination-based mixging study.

Question 23

What is vonvendi?

Answer 23

recombinant VWF

Question 24

What is the defect in Bernard-Soulider syndrome?

Answer 24

loss of function mutation in Gp1b-alpha that leads to reduced adhesion

Question 25

What is missing in grey platelet syndrome?

Answer 25

alpha granules

Question 26

What is the defect in Glanzmann thrombasthenia?

Answer 26

Gp2a3b

Question 27

What is the HbS goal upon completion of red cell exchange?

Answer 27

<30%