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34 Cards in this Set

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How is primary hemostasis best evaluated (2)?
Platelet function tests
vWF studies
What is Osler-Weber-Rendu HHT?
HHT = Hereditary Hemorrhagic Telangiectasia

Autosomal dominant disorder

Telangiectasias on face, mucous membranes, liver, brain

Frequently complicated w/ epistaxis and GI bleeds
What is the most common inherited bleeding disorder?
Von Willebrand Disease
How is Von Willebrand Disease inherited?
Autosomal dominant
Differences in Von Willebrand Disease types (1 through 3)
Type 1 - decrease in vWF

Type 2 - defective vWF
(1 defective gene (heterozygote))

Type 3 - NO vWF
(2 defective genes (homozygote))
How are vWF and Factor VIII related?
vWF carries Factori VIII

SO, in severe vWD, there is a very low Factor VIII level
Ranking of blood types according to baseline levels of vWF
AB > A > B > O
What is 1st line therapy for VWD?

What does it do?
Desmopressin

Releases vWF stores from endothelium

NOTE: NOT all pts. respond
NOTE: tachyphylaxis after 2-3 doses
What drug is used in supportive therapy for vWD?

What does it do?
Amicar

Stabilizes clots
What treatment is given to desmopressin non-responders?
vWF concentrates
In addition to the liver, what else makes Factor VIII?
Endothelial cells
In addition to the liver, what else makes Factor V?
Megakaryocytes
In addition to the liver, what else makes Factor III?
Monocytes
Smooth muscle
Endothelium
Which clotting proteins are K-dependent (6)?
Factors II, VII, IX, X
Prothrombin
Protein C
What is the role of Vitamin K in clotting?
Involved in carboxylation of Gla residues

Gamma-carboxy Gla is required to bind Calcium
Which type of hemophilia is NOT sex-linked?
Hemophilia C

It is autosomal recessive
How are Hemophilia A & B inherited?
X-linked Recessive
Hemophlia A involves abnormalities of what factor?
Factor VIII
Hemophilia B involves abnormalities of what factor?
Factor IX
What is the most common type of hemophilia?
Hemophilia A (80 - 95%)
Comparisons of severities of hemophilia
---Mild---
Very rare spon. bleed, bleed w/ MODERATE trauma

---Moderate---
Unusual spon. bleed, bleed w/ MINOR trauma

---Severe---
Common spon. bleed, excessive bleeding w/ MINIMAL trauma
What is the dominant clinical feature of hemophilia?
PROLONGED bleeding into joints, muscles, soft tissue
Abnormalities of what are involved in liver coagulopathy?
(clotting factors)
F VII first, F VIII, Fib last
3 major problems assoc. w/ DIC
Thrombosis of small vessels causes end organ ischemia

Fibronylisis of thrombuses causes reperfusion injury

Consumption of platelets and clotting factors cause bleeding
Can a clotting factor deficiency be overcome in a mixing test?
YES
Can presence of a coagulation inhibitor be overcome in a mixing test?
NO
You have an isolated, prolonged PT

If 50:50 mixing corrects the PT, what could be wrong (3)?
This means that there was a clotting factor deficiency

Factor deficiency (Factor VII)
Vit K deficiency
Liver disease
You have an isolated, prolonged PT

If 50:50 mixing CANNOT correct the PT, what could be wrong (2)?
Something is inhibiting the extrinsic pathway

Factor VII inhibitor
Lupus anti-coag
You have an isolated, prolonged aPTT

If 50:50 mixing corrects the aPTT, what could be wrong?
Factor Deficiency
(Factors XI, IX, VIII)

NOTE: think about vWF if VIII is low
NOTE: if there is NO bleed, think about Factor XII
You have an isolated, prolonged aPTT

If 50:50 mixing CANNOT correct the aPTT, what could be wrong(3)?
Something is inhibiting the intrinsic pathway

Factor inhibitor (VIII, IX, XI)
Heparin
Lupus anti-coag
You have a prolonged PT AND aPTT

If mixing corrects neither, what could be the problem (2)?
You have inhibition of the COMMON coag pathway

Factor V, X, II inhibitor
Lupus anti-coag
You have a prolonged PT AND aPTT

If mixing corrects both, what could be the problem?
You have a deficiency of common pathway factors
(V, X, II)
What is cryoprecipitate?

What does it contain?
Precipitate of frozen plasma

vWF, Factors VIII, XIII
Fibrinogen, Vitronectin, Fibronectin
Hemophilia C is assoc. w/ what Factor deficiency?
Factor XI