Hematology

Question 1

Factor XI deficiency (hemophilia C)

Factor XII deficiency

Factor XIII deficiency

Answer 1

XI = ashkenazi jews; recombinant XI not available in US; give FFP

XII = prolongs PTT but no clinical consequences

XIII = normal PT and PTT; recombinant XIII

Question 2

AC that targets Xa

Answer 2

Target Xa
	ApiXaban
	EdoXaban
	RivaroXaban
	FondaparinuX

Question 3

AC that targets thrombin

Answer 3

Target IIa (thromBin)
ArgatroBan
Bivalirudin
DaBigatran

Question 4

Heparin mechanism

Answer 4

Targets both Xa and thrombin
Heparin
LMWH (Xa > thrombin)

Question 5

Clinically significant APL ab are one of the following on 2 or more occastions 12 weeks apart…

Answer 5

1) positive LA test
2) ACL IgG and IgM with titer > 40 units
3) Anti-B2 GP igG or IgM with titer > 40 units

Question 6

Duration of treatment for provoked and unprovoked clot

Answer 6

Provoked → 3 months

Unprovoked → 6 months (prolonged if life-threatening PE, etc)

Question 7

PPX AC during pregnancy if thrombophilia

Answer 7

High risk thrombophilic state → therapeutic AC during preg
(ATIII deficiency, homozygous for FVL or PT gene mut or compound heterozygote FVL or PT gene mutation)

Lower risk thrombophilic state → monitor during preg w/o AC however AC postpartum if C-section
(Heterozygote FVL or PT gene mutation or protein S/C def)

Question 8

pregnancy thrombocytopenia - gestational thrombocytopenia versus ITP

Answer 8

gestational thrombocytopenia - usually plts > 70 and happens 2nd-3rd trimester

ITP - usually starts 1st trimester

Question 9

PNH

Answer 9

Absence of two GPI-anchored proteins, CD55 and CD59 → uncontrolled complement activation → hemolysis etc

Clonal hemotopoeitic stem cell disease - hemolytic anemia, smooth muscle contractions*, thrombosis, bone marrow failure, hemoglobinuria (intravascular process)

Question 10

atypical HUS

Answer 10

Uncontrolled complement activation 2/2 loss of function variants in complement regulatory proteins OR gain of function variants in complement activation factors

Something triggers them leading to aHUS

Question 11

Beta thal minor versus intermedia vesus major

Answer 11

Thal minor or silent carrier (depending on any anemia or not): B+/B

Thal minor: B0/B

Thal intermedia: B+/B+

Thal major (cooley anemia: B0/B0

Question 12

alpha thal

Answer 12

aa/a- = silent carrier, no anemia

a-/a- or aa/– = trait, mild anemia

–/a- = hg H disease, intermediate anemia

–/– = hydrops fetalis, lethal