Flashcards in Hemostasis Defects Deck (66)
When is Factor XI deficiency most commonly diagnosed?
What test time is usually prolonged in Factor VII deficiency?
What is the inheritance pattern of Factor VII deficiency?
Autosomal disease: Homozygous severe but heterozygous still affected.
What are the two functions of von Willebrand protein?
1. Adhere platelets to exposed collagen at the wound site.
2. Carrier of Factor VIII
What two tests are prolonged in von Willebrand's Disease?
What is type I von Willebrand's disease?
Deficiency of normal von Willebrand protein
What is type II von WIllebrand's disease?
Presence of an abnormal protein
DDAVP (arginine vasoprressin is very effective in treating which type of von Willebrand's disease?
What is the inheritance pattern of von Willebrand's disease?
What are some symptoms of von Willebrand's disease?
Bleeding from mucosal mebranes, and nose bleeds, GI bleeds and menorrhagia
They also bleed after surgery
Acquired Factor VIII inhibitor is an extremely rare acquired factor inhibitors seen in postpartum or advanced age patients.
How does this acquired disease present?
What test is prolonged?
-Soft tissue and muscle bleeding, and usually marked hematomas of their skin or mucosal bleeding
-Abnormal PTT only. Which will not correct after two hours of incubation.
What is the prognosis for Aquired Factor VIII inhibitor?
Although the disease has a 25% mortality due to bleeding it has an EXCELLENT long-term prognosis since most patients respond to immune suppression.
Name some very rare acquired inhibitors that can be seen.
Inhibitors to von Willebrand protein
Factor II inhibitors
Factor V inhibitors
Where are most coagulation factors synthesized?
What are the factors that are especially lowered due to liver disease?
What tests are prolonged in liver disease?
Relatively less prolonged PT
Thrombin time may also be prolonged
In liver disease you should always make sure that there is not a component of ____________ deficiency
Vitamin K is obtained from the diet. What two circumstance cause vitamin deficiency?
No oral intake and broad spectrum antibiotics
A 16 year old patient is brought into the emergency room by his parents. He is unconscious and his parents report that he suffers from depression and that they found a box of d-Con by his bed. They show you the box that has an upside-down rat on it. Based on your knowledge of rat poison you know that this patient will need what type of therapy? Besides charcoal and gastric lavage blah blah blah
Vitamin K therapy for many months
What two tests are decreased in disseminated intravascular coagulation?
Fibrinogen level is decreased
Platelet count is low
Fibrin cleavage products, fibrin degradation products and D-dimer can be measured in DIC. These products can inhibit two tests. Which two tests?
PTT >>>it being prolonged
TT>>>>.it being prolonged.
What two factors are consumed in DIC?
Factor VIII and Factor V
How is DIC distinguished from liver disease just based on the labs?
Liver disease PT is prolonged a lot more than PTT
DIC: PTT is increased relatively much more than the protime
Formation and propagation of clot within the vasculature
Thrombosis occurs due to some combination of what three factors?
Vessel wall injury
What factors create a hypercoagulable state?
Chronic damage to vessel walls
Excess of procoagulant factors
Deficiency of anticoagulant facotrs
Name four risk factors for thrombosis
1. A thrombotic episode in the absence of a defined precipitating condition
2. Recurrent episodes of throbosis, or throbosis at an early age and is otherwise health
3. A severe, life threatening thrombosis, or thrombosis at an unusual site
4. Family history of thrombosis
Is it appropriate to search for an underlying hemostatic defect that can contribute to a "hypercoagulable state"?
Lupus anticoagulant is
rare or common?
congenital or acquired?
Common acquired abnormality