Hematology Flashcards
Reversal agent for dabigatran
idarucizumab (Praxbind)
Reversal agent for apixaban/rivaroxaban
andexanet alfa (Andexxa)
Indications for rFVIIa
patients with congenital hemophilia A or B with inhibitors, patients with acquired hemophilia, congenital factor VII deficiency, Glanzmann thrombasthenia w/platelet refractoriness
Condition with thrombocytopenia and granulocyte dohle bodies
May-Hegglin anomaly (MYH9 gene)
Prophylaxis needed with eculizumab
meningococcal (antibiotics or vaccine)
What is passenger lymphocyte syndrome?
Alloimmune hemolysis of host erythrocytes by antibodies produced by donor lymphocytes; occurs in solid organ transplant recipients
What is caplacizumab
anti-vWF antibody; used for treatment of TTP in combo with immunosuppression and PLEX
Which subtypes of vWD may be inherited in a recessive manner?
type 2N and type 3
What is the defect in type 1 vWD?
Partial quantitative deficiency of vWF (usually low secretion or increased clearance)
What is the defect in type 3 vWD
virtually complete deficiency of vWF
What is the defect in type 2A vWD?
Defect in multimerization or increased cleavage of multimers by ADAMSTS13
What is the defect in Type 2B vWD?
Increased affinity for platelet GP1b
What is the defect in Type 2M vWD?
Decreased vWF mediated platelet adhesion
What is the defect in Type 2N vWD?
Markedly decreased binding affinity for FVIII
Lab findings in Type 1 vWD?
VWF:AG <30, VWF:RCo <30, Ratio RCO:Ag >0.6, normal or slightly decreased FVIII, Normal Multimers, Normal RIPA
Lab findings in Type 2A vWD?
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
Lab findings in Type 2B vWD?
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
Lab findings in Type 2M vWD?
VWF:AG 30-200, VWF:RCo <30, Ratio RCo:Ag <0.6, normal or slightly decreased FVIII, normal multimers, decreased RIPA
Lab findings in Type 2N vWD?
VWF:AG 30-200, VWF:RCo 30-200, Ratio RCo:Ag >0.6, decreased FVIII, normal multimers, normal RIPA
Lab findings in Type 3 vWD?
VWF:AG <5 VWF:RCo <5 Ratio RCo:Ag N/A, very decreased FVIII, absent multimers, absentRIPA
What is platelet-type vWD?
rare disorder caused by gain of function mutation in GpIB that mimics vWD type 2B
How is the diagnosis of platelet type vWD confirmed?
Enhanced binding of exogeneous VWF substrate to patient’s platelets in a ristocetin-induced platelet agglutination-based mixging study.
What is vonvendi?
recombinant VWF
What is the defect in Bernard-Soulider syndrome?
loss of function mutation in Gp1b-alpha that leads to reduced adhesion
What is missing in grey platelet syndrome?
alpha granules
What is the defect in Glanzmann thrombasthenia?
Gp2a3b
What is the HbS goal upon completion of red cell exchange?
<30%
