Treatment for Bleeding Disorders

Question 1

What does fresh frozen plasma contain?

Answer 1

all of the components of blood except for platelets, RBCs, and factor VIII.

contains plasma proteins for

• coagulation (except calcium)
• fibrinolytic system
• complement systems
• maintaining oncontic pressure
• modulation of immunity
• fats, carbs, mineral

Question 2

How long does FFP need to thaw before you can use it?

Answer 2

-takes at least 30minutes to thaw, so NOT for IMMEDIATE use.

Question 3

When is FFP appropriate for use?

Answer 3

• 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.

Question 4

Can you have a transfusion rxn to FFP?

Answer 4

Yes, it needs to be ABO compatible, dont have to worry about Rh factor.

Question 5

What does cryoprecipitate come from?

Answer 5

-comes from FFB, you thaw FFP and the precipitate from the bottom is drawn off.

Question 6

What is in cryoprecipitate?

Answer 6

-fibrinogen, factor VIII (but not much), vWF, Factor XIII, and fibronectin

Question 7

When would you use cryoprecipitate?

Answer 7

• vonWillebrand disease if DDAVP is ineffective and specific vWF concentrates are unavailable
• hypofrinogenemia (DIC)
• Factor VIII deficiency

Question 8

How is cryoprecipitate supernatant plasma (CSP) made?

Cryoprecipitate supernatant plasma aka?

Answer 8

• the left over plasma that was used to make cryoprecipitate now becomes CSP. (the liquid portion that didnt get spun out)
• aka: cryo-reduced plasma

Question 9

When can you use cryoprecipitate supernatant?

Answer 9

-in tx of TTP (this is better than using plasma because it lacks the vWF)

Question 10

What are some reasions you’d give platelet transfusion?

Answer 10

• thrombocytopenia, when there is a PRODUCTION problem and the platelets are extremely low.
• ITP, cancers,
• do not give in conditions where there is concurrent thrombosis for fear of worsening the clot. (TTP, DIC, HIT)

Question 11

How many units of platelets should be transfused?

How quickly should platelet counts rise?

Answer 11

• A LOT! it takes 6U to increase the platelet count by 30,000.
• counts should rise within 1-60min of transfusion

Question 12

When is recombinant factor VIIa used?

Answer 12

for those w/ Hemophilia A and B who have developed abys to their specific factors (A-VII, B-IX)

• tx of warfarin related intracerebral hemorrhage
• Factor VII deficiency

Question 13

When would you give Factors VIII and IX?

• how often can this be given?
• what are the SE of giving this?

Answer 13

• VII: Hemophilia A
• IX: Hemophilia B

Should not have more than one dose in 24hrs

They may develop abys or “inhibitors” to the clotting factors, this is dose dependent. (the higher the dose of factor you give them the more likely they are to develop abys to that factor.

Question 14

Hemophilia C is what factor deficient?

-whats unique about hemophilia C that differentiates it from A or B?

Answer 14

• deficient of factor XI
• unlike hemophilia A or B, most patients do not suffer from spontaneous bleeding but are at risk of bleeding following trauma or surgery.

Question 15

What are some major side effects of factor concentrates?

Answer 15

• thrombus formation
• hypersensitivity rxn
• development of abys to the factor (once they develop abys they can no longer have the factors)

Question 16

What is Autoplex T? What disorder is it used in?

What is it made up of?

When is it used?
Off label use?

Answer 16

• Anti-inhibitor coagulant complex
• used in Hemophilia A
• contains nonactivted II, IX, X, V and VIIIa
• used in patients with abys or “inhibitors” to factors VIII and are bleeding or about to undergo surgery.

Off label use: case reports of life threatening bleeding of patients on Pradaxa.

Question 17

What is Aminocaproic acid?
AKA
used for?

Answer 17

synthetic inhibitor of plasminogen-plasmin system (antifibrinolytic agent)

AKA: Amicar

used for acute bleeding–bleeding post dental extraction in hemophilia A/B and vonwillebrands dz, epistaxis, Menorrhagia

Question 18

Aprotinin

• when is this used?
• derived from what animal?

Answer 18

• hemostatic agent used during CABG surgery when at high risk of bleeding and no other hemostatic agent can be used.
• derived from cow lung tissue

Question 19

Desmopressin Acetate

• aka
• what does it do?
• administration routes
• in the tx what what two diseases is this drug most often used?

Answer 19

-aka DDAVP

• synthetic analogue of vasopressin (ADH), increases circulating VIII from endothelial storage sites
• IV, SQ, or nasal spray
• Hemophilia A and vonWillebrand disease

Question 20

SE of DDAVP

Answer 20

• water retention, hyponatremia (seizures, death)

- limit dose to once daily to avoid the development of tachyphylaxis(too much medication so it no longer works)