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15 Cards in this Set

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18 y/o female about to undergoing neurosurgery. she has prolonged PTT.

what factor assays do you request?
A. FXII, FXI, FVIII (F12, F11, F8)
B. FII, FV, FVII (F2, F5, F7)
C. FX, FVII, FII (F10, F7, F2)
D. FVIII, FIX, FXI (F8, F9, F11)
E. FXIII, FIX, FVII (F13, F9, F7)
D.
why don't you need to know factor 12?
you don't need to know factor 12 before surgery bc low factor 12 NEVER causes bleeding. so that's why we just want to see factor 8,9, and 11.
Results:
Factor 8 43% [47-169%]
Factor 9 106% [67%-141%]
Factor 11 66% [48%-139%]

Conclusion:
The mild prolongation of the PTT is explained by a low factor 8 and a low normal factor 11.
Work-up ended. Results were reported.

What would you do next?

a. None - Clear the patient for surgery.
b. Transfuse plasma before surgery.
c. Do further study.
d. Transfuse platelets.
e. Administer factor VIII.
C.
afterwards, a von Willebrand panel was ordered:
Factor 8: 43% (low)
Von Willebrand factor antigen: 25%
Von Willebrand factor activity: 15% (low; <30%)
Multimer: Normal distribution

Dx?
von Willebrand disease (type 2N or 3)
most common bleeding disorder in the US
von Willebrand disease
what is the 2 most common complaints for a pt with vWD
recurrent nosebleed or severe menorrhagia
diff the clinical features of vWD and hemophila:

PT
PTT
PFA
FVIII
vWF
Feature vWD Hemophilia

PT Normal Normal

PTT Normal or Prolonged
prolonged

PFA Prolonged Normal

FVIII Decreased Decreased

vWF Decreased Normal
most common type of vWD
type 1
3-month history of worsening petechiae on her face and extremities.
Intermittent bloody stools for the past 2 weeks.
She received vaccines 2 days prior and had persistent oozing from the injection site.
Father has nosebleed 3 times a day.
Hemoglobin 11.0 g/dL (ok), hematocrit 33.2%, platelet 630,000/mm3 (high)
AST 23, ALT 19, total bilirubin 0.6 mg/dL(normal liver fxn)
PT 14.3 sec, PTT 34.1 sec, fibrinogen 347 mg/dL(all normal for her age), thrombin time 15.9 sec, D-dimer 1.08 mg/mL, PFA was abnormal!!

Epinephrine - 0%
ADP – 0%
Collagen – 0%
Arachidonic acid – 0%
Ristocetin – 70% (normal)

Glycoprotein (GP) Ib: normal
GPIIb/IIIa: Abnormal


What's the diagnosis?

a.Bernard-Soulier disease
b.Glanzmann’s thrombasthenia
c.Storage pool deficiency
d.Immune thrombocytopenic purpura (ITP)
e.Thrombotic thrombocytopenic purpura (TTP)
B.
congenital platelet dysfunction that:
1)lack of alpha-granules and/or dense bodies
2) lack of GPIa
3) lack of GPIIb/IIIa
1) storage pool deficiency (defective storage space)

2) bernard-soulier syndrome (defective adhesion)

3) glanzmann's thrombasthenia (defective aggregation)
tx of glanzman's thrombasthenia
platelet transfusion
just study this to get oriented with which dz is where...ok?
ok!
type 1 and 3 vWD are associated in that both are:

a. reduced quantity of circulating vWF
b. autosomal recessive disorders
c. defects in vWF where the multimers formed are abnormal
d. none of the above
A. type 1 is much more mild compared to type 3, which is associated with extremely low levels of functional vWF
deficiency of vWF gives rise to a secondary decrease in factor:

a. V
b. VII
c. VIII
d. XII
C. this is what prolongs the PTT in vWD type 1 and 3
Among the following, which is not contained in cryoprecipitate?

a. Fibrinogen
b. Factor V
c. Factor VIII
d. Factor XIII
e.von Willebrand factor
f. Fibronectin
B.