clotting factor disorders Flashcards
a genetic mutation that increases a person’s risk of developing abnormal blood clots
considered the most common inherited blood clotting disorder
it results from a point mutation in the factor V gene which leads to an amino acid substitution that renders factor V resistant to inactivation by activated protein C (APC)
Factor V Leiden
how to detect presence of Factor V Leiden
APC resistance assay that assay that assesses the ability of protein C to inactivate factor V
Symptoms include prolonged bleeding after surgery, dental procedures, or trauma, as well as frequent nosebleeds, easy bruising, and bleeding gums.
- normal PT
- prolonged aPTT
factor XI
factor XI treatment
FFP
It’s produced in the liver and circulates in the bloodstream in an inactive form until a blood vessel is damaged. When that happens, it separates from von Willebrand factor and becomes activated. It then interacts with factor IX, which sets off a chain of reactions that form a blood clot.
Unexplained bleeding after minor injuries, surgeries, or dental procedures
Easy bruising
Slow wound healing
Joint pain and stiffness
Heavy menstruation
Severe low back pain
- prolonged PT
- normal aPTT
factor VIII
factor VIII deficiency treatment
Desmopressin: Helps the body release factor VIII that’s stored in blood vessel linings
Factor VIII concentrates: Can be given at home at the first signs of bleeding
It’s also known as Christmas factor, and a deficiency causes hemophilia
B
Made in the liver and circulates in the bloodstream in an inactive form
Activated by factor XIa in response to injury
Symptoms:
Bleeding into joints, blood in urine and stool, bruising, nosebleeds, and prolonged bleeding from cuts
- normal PT
- prolonged aPTT
factor IX
factor IX deficiency treatment
Injections of factor IX to help control bleeding
most common inherited
bleeding disorder
Symptoms:
nosebleeds in children, easy
bruising, bleeding gums, and postsurgical bleeding.
Gynecologic problems are especially common. Most women experience heavy menstrual bleeding and menorrhagia
endometriosis (likely related
to backflow of menses), miscarriages
(unclear mechanism), and postpartum hemorrhage are more common in women
von Willebrand
Disease
how to diagnose von willebrand disease
Results of the Platelet Function
Analyzer-100 (PFA-100®) are usually
prolonged.
The PT is normal and the aPTT may be normal or slightly prolonged, because the factor VIII level may be slightly low
a life-threatening complication that can occur when a patient is exposed to heparin, an anticoagulant. HIT occurs when the body produces antibodies that attack platelet factor 4 (PF4) in complex with heparin, which activates platelets and can lead to thrombosis.
heparin induced thrombocytopenia
how to diagnose heparin induced thrombocytopenia
look for heparin/PF4 complex antibodies
- ELISA
- serotonin release assay (gold standard)
Pain, swelling, redness, or tenderness in an arm or leg
Sudden sharp chest pain
High blood pressure
Feeling faint, dizzy, or light-headed
Rapid heartbeat
Coughing and wheezing
Feeling out of breath
Excessive sweating
symptoms of heparin induced thrombocytopenia
treatment for heparin induced thrombocytopenia
- Discontinuing heparin
- Treating with an alternative anticoagulant (agatroban)
- Avoiding platelet transfusions.
consumptive coagulopathy in
which clotting factors as well as
platelets get consumed due to
uncontrolled intravascular coagulation.
When the body’s clotting system becomes overactive, it produces numerous small clots that can obstruct blood flow to organs, causing tissue damage; simultaneously, the excessive clotting depletes platelets and clotting factors, leading to bleeding tendencies.
In most cases procoagulant tissue factor, a lipoprotein which is not normally exposed to blood start the coagulation. It gains entry to blood.
disseminated intravascular coagulation (DIC)
