Coagulation Disorders-COMP Flashcards
slide 10 Von willebrand disease
Factor VII deficiency
Von willebrand disease is the
most common INHERITABLE coagulation disorder
Hemophillia A Treatment
Plasma derived (may developed antibodies ) and recombinant factor
1 unit of factor VIII is equal to
amount found in 1Ml of plasma
For surgery you need_____concentrate
100% of concentrate
Each unit of factor of VIII per kg of body weight raises plasma by
2%
Tx for hemophilliac going through surgerry
Factor VIII levels 1 hour prior to surgery 100% then 50% wound healing begins, then 30% until complete healing
Dose of Factor VIII
Concentrate 50 units/kg then dose q12h
______infuse over 15 minutes may be given to treat mild bleeding episodes
• DDAVP 0.3-0.4ug/Kg
Intranasal treatment for DDAVP what is recommended?
•Recommended to give a test dose prior to using intranasal DDAVP
Most common side effect of DDVAP
FACIAL FLUSHING
DDVAP also increase
Plasminogen level
When do you suspect inhibitors
when calculated dose does not result in desired increase in factor level.
Scenario: factors VIII inhibitors
FEVA (factor inhibitors treatment ) Bypass factor VIII
Hemophillia A is due to a deficiency in what clotting factors factor?
Factor VIII deficiency
Hemophillia B is due to a deficiency in what clotting factors factor?
Factor IX deficiency
How are patients with Hemophilia classified
Clinical bleeding with the degre of deficiency
Severe hemophilliacs
Moderate hemophilliacs
Mild hemophilliacs
Most commonly affected for hemophillia A
Males affected; females are carriers
Hemophillia A presentation
joint or muscle hemorrhage with prolonged bleeding after trauma or surgery.
Hemophillia A Diagnosed
normal PT and abnormal PTT (unique to intrinsic factors VIII, IX, XI and XII)
Lab test for hemophillia A
Test for Von willebrand disease
Speficic factor assay for factor VIII or IX
Recommended treatment for Hemophillia A
Severe or moderate: Cryoprecipitate or FACTOR VIII concentrate
2 sources of factor VIII concentrate
Plasma derived
Recombinant Factor VIII
1 unit of factor VIII is the amount found in
1 pooled plasma
Treatment plan for hemophilliacs undergoing surgery? Factor level
Desired factors VIII levels 1 hour prior to surgery 100%
then 50% until wound healing begins then 30% until complete wound healing
Treatment plan for hemophilliacs undergoing surgery? Dose factor VIII concentrate
50 units/kg Then dose 12 hr
Treatment plan for hemophilliacs undergoing surgery?Perioperative and postop
Plan must be in place preop; evaluation for inhibitors crucial prior to elective surgery
What is cryoprecipitate?
•Rich in factor VIII, fibrinogen, and von Willibrand’s factor
Each bag of cryoprecipitate contains_______ Potency can vary from batch to batch
70-100 units of factor VIII activity per 10-20ml bag.
What is Desmopressin Acetate (DDAVP) ?
Used to treat mild factor VIII deficiencies.
Main action of DDAVP
transiently increases factor VIII and von Willibrand’s factor levels and shortens prolonged bleeding
DDAVP is a
synthetic analog of the antidiuretic hormone vasopressin
Dose of DDAVP for mild bleeding
0.3-0.4 mcg/Kg infuse over 15 minutes may be given to treat mild bleeding episodes
Inhibitors : Treatment can be approached by different methods •
Reduce inhibitor levels with immunosuppresant therapy (cyclophosphamide), gamma-globulin, plasmopheresis
Or by tolerance with high dose OR frequent administration of factor
B vs Bm The exception is in hemophilia Bm in which
Both the PT and APTT are prolonged; Hemophillia B has normal PT
What is the treatment of choice for Hemophilia B?
High purity factor IX plasma concentrate
High purity factor IX plasma concentrate replace older products, known as ______ witch also contained _______________dependent cofactors responsible for thromboembolic complications associated with their use.
•Replaced older products, known as PCC witch also contained vitamin K dependent cofactors responsible for thromboembolic complications associated with their use.
Each unit of factor IX infused per Kg increases factor IX plasma levels
1%.
Classification of Von willebrand
quantitative or qualitative of von Willibrand’s factor
Von willebrand disease results in both
both abnormal platelet function and defective plasma clotting.
von Willebrand’s disease and it’s variants are defined by the type of abnormality in vWF: Factor VIII acAvity is usually low depending on the degree of vWF deficiency, however it may approach normal in some paAents.
quantitative, qualitative or both. •
Diagnosis: VWF activity is measured by
1)the bleeding time and 2)ristocetin induced platelet aggregation (RIPA). •
What is the recommended treatment for mild von willebrand disease? Goals?
•Goals of therapy are to improve factor VIII levels, and to improve bleeding time. •
What is the recommended treatment for mild von willebrand disease?
Cryoprecipitate • The majority of commercial concentrates used to treat hemophilia A are not suitable for vW disease because they lack high molecular weight multimers, and therefore do not correct the bleeding time. •
What is the recommended treatment for mild von willebrand disease that can achieve clinical hemostasis
Humate -P, Koate-HS, and Koate-HP have been reported to achieve clinical hemostasis prior to surgery and can be used in place of cryoprecipitate
_________ dose and meds: usually induces a dose dependent increase in all factor VIII activity, with both factor VIII and vWF increasing four to six-fold
Intravenous DDAVP 0.3 to 0.4ug/Kg over 15 -30 minutes
How long does the response to DDAVP lasts _____and should be repeated
The response usually last 4-8 hours. • The dose should be repeated in 12 hours. •
When should DDAVP not be used?
should not be used before the type of vW disease is known
DDVAP should not used before you know the type of DDVAP because?
In type 2B VW disease DDAVP can cause platelet aggregation and severe thrombocytopenia
Use high molecular weight vWF because
they have the Highest RISTOCETIN FACTOR, most efficient for platelet adhesion
