Treatment options for bleeding disorders Flashcards
What does FFP contain?
all the components of normal blood except for platelets and RBCs.
Specificically FFP contains plasma proteins for the function of what?
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Coagulation (except calcium) Fibrinolytic system Complement systems Maintaining oncotic pressure Modulation of immunity As well as fats, carbohydrates and minerals
How long does FFP take to thaw?
Takes at least 30 minutes to thaw so not for IMMEDIATE use
What is not in FFP?
So who is this not a treatment option for?
Factor 8. because it doesnt last long enough (only seconds to minutes even with VWF) to be in FFP
Hemophelia A patients
FFP transfusions are appropriate for which syndromes?
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- Clotting factor deficiencies if no clotting factor concentrates are available
- Reversal of warfarin
- Liver disease/replace clotting factors for potential invasive procedure
- DIC
- TTP
- Massive RBC transfusion, may need to replace some clotting factors
Can you have a transfusion reactions with FFP?
Why?
YES! needs to be ABO compatible but dont have to worry about Rh
Cryoprecipitate is made from what and how?
What are the components of cryoprecipitate?
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Thaw FFP and the precipitate from the bottom is drawn off. This contains Fibrinogen, Factor VIII (not much factor 8), vWF, Factor XIII, fibronectin
What is cryoprecipitate indicated for?
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vonWillebrand disease If DDAVP is ineffective and specific vWF concentrates are unavailable
Hypofrinogenemia (DIC)
Factor XIII deficiency
What is Cryoprecipitated supernaturant plasma. And whats another name for it?
cryo-reduced plasma is the left overs from Cryo.
The left over plasma that was used to make cryoprecipitate now becomes CSP.
How can we use this for treatment options?
Can use this as an alternative to FFP in the treatment of TTP (plasma exchange).
Think about what this is missing and explain why might this be a good idea?
Indications for platelet transfusions
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In what situations would we not give platelet transfusion?
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- Thrombocytopenia when there is a production problem and the platelets are extremely low.
- ITP, cancers, etc.
Do not give in conditions where there is concurrent thrombosis for fear of worsening the clotting. (TTP, DIC, HIT)
How many units should be transfused?
When should the count rise after transfusion of platelets?
A lot!
It takes 6 U to increase the platelet count by 30,000
Counts should rise within 10-60 min of transfusion
Recombinant Factor VIIa is indicated for what?
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- Hemophilia A (low factor 8) and B (low factor 9) patients who develop antibodies to their specific factors can use factor VIIa
- Treatment of warfarin related intracerebral hemorrhage
- Factor VII deficiency
Factor VIII and IX are indicated for what?
What is a complication that could come from this?
Hemophilia A and B for prevention and treatment of bleeding
The development of antibodies or “inhibitors” to the clotting factors is dose dependent. high dose = greater
VERY VERY expensive
Whats the limit on dosing factor VIII and IX in a 24 hour period?
once only
What is factor XI deficiency most common in?
Most common with Jewish ancestry
How does factor XI deficiency differ from hemophilia A and B?
Unlike classic hemophilia A or B, most patients with severe deficiency do not suffer from spontaneous bleeding but are at risk of bleeding following trauma or surgery.
What are the major side effects for factor concentrates?
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Thrombus formation
Hypersensitivity reaction
Development of antibodies to the factor
Autoplex T (Anti-inhibitor coagulant complex) is used for what?
What does it contain?
Why is it indicated?
Off label use for Autoplex T?
Hemophilia A
Contains nonactivated II, IX, X, V and VIIa
Patients with antibodies or “inhibitors” to factor VIII and are bleeding or about to undergo surgery
case reports of life threatening bleeding of patients on dabigatran (Pradaxa)
What is Aminocaproic acid (ACA) MOA for and what is another name for it?
Synthetic inhibitor of plasminogen-plasmin system
Antifibrinolytic agent (will cause clot to stay where its at and prevent other clots while body works on breaking down the clot)
FDA label use “acute bleeding”
Helps stabilize clots by inhibition of fibrinolysis
What is Aminocaproic acid (ACA) indicated for?
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Bleeding post dental extractions in hemophilia A/B and vonWillebrand dz
Epistaxis
Menorrhagia
ACA has many “off label” uses that may be controversial.
What are they?
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- Control and prevention of oral bleeding with a history of coagulation disorders
- Treatment of traumatic hyphema
- Control of bleeding with severe thrombocytopenia (ex: aplastic anemia)
- Prevention of perioperative bleeding with cardiac surgery
- GU bleeding post prostate surgery or obstetrical surgery
What is aprotinin indicated for?
Hemostatic agent used during CABG surgery when at high risk of bleeding and no other hemostatic agent can be used. (allergy)
What is desmopressin acetate?
What does it do?
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Synthetic analogue of vasopressin (ADH)
- Increases circulating VIII from endothelial storage sites
- Promotes the release of vWF from endothelial storage sites
DDAVP
What diseases is DDAVP used for?
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Hemophilia A and vonWillebrand disease
Which types of hemophilia is DDAVP used for?
When do we use it to treat VWD?
Treatment of choice for mild to moderate bleeding in hemophilia A
Treatment of bleeding or prophylaxis prior to procedures in vonWillebrand’s disease
How is DDAVP usually administered?
IV, SQ or nasal spray (most common)
Side effects of DDAVP? 3
How many doses are we limited to daily?
- Water retention,
- hyponatremia (seizures, death)
- have too much of the medication and it doesn’t work anymore
Limit dose to once daily to avoid the development of tachyphylaxis
