Products and Factors for Bleeding Flashcards

1
Q

What are the two types of platelet donors

A

‘Random Donor’ Platelets
Apheresis single donor platelets

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2
Q

Compare Random donor vs apheresis single donor platelets
(3)

A

Random -> single donor units containing at least 45-85 x10^9 per donation. Usually pooled in 4-6 unit bags and issued to the hospitals. Units would have approx 250-300 mls plasma

Apheresis -> contain at least 240 x 10^9 platelets per unit. Suspended in 300mls approximately of male donor plasma.

Both ABO grouped if possible

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3
Q

Why are apheresis donors men

A

Men tend to not have HLA antibodies

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4
Q

How are apheresis platelets made

A

Take off 75% of plasma and replace with platelet additive solution

i.e. platelets suspended in 25% male plasma solution

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5
Q

Compare the shape of resting platelets vs activated platelets

A

Resting platelets are smooth and disc shaped

Activated platelets have an irregular shape with many protruding pseudopodia

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6
Q

What is the platelet swirl test

A

If platelets have activated e.g. if bacteria present in plasma then they won’t swirl, you will be able to see clumps in pack

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7
Q

How are platelets processed
(4)

A

Agitated during storage to prevent unacceptable platelet aggregation

All platelet products in Ireland are ‘leucodepleted’ by filtration

All platelet products are irradiated since 2006

CMV antibody tested (donor) for appropriate immunosuppressed patients e.g. neonates, BMT, chemotherapy

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8
Q

Why do we irradiate platelets and not rbc packs

A

There is no shortening on the life span of platelets when irradiated unlike rbcs

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9
Q

Why do we test for CMV in healthy patients and how do we remove it?
(4)

A

CMV is always there in your system

CMV hides itself in wbcs

When you irradiate the platelets you remove the wbcs and thus the CMV (pack deemed CMV negative but not deffinite)

We can test for CMV antibodies but this is only done in special cases e.g. for neonates

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10
Q

Who might need a platelet transfusion?
(6)

A

Bone marrow failure e.g. aplastic anaemia, leukaemia

Chemotherapy

Acute DIC -> platelets consumed in clots

Dilutional thrombocytopoenia -> massive transfusion

Congenital platelet adhesion and aggregation defects

Neonatal alloimmune thrombocytopoenia (NAITP)

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11
Q

What are two congenital platelet adhesion and aggregation defects

A

Bernard Soulier and Glanzmanns disease

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12
Q

What is NAITP

A

Neonatal alloimmune thrombocytopoenia

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13
Q

What response is there when a patient receives a platelet transfusion?
(3)

A

One unit of random donor platelets should raise the count by 5-10 x10^9/L

Anticipate platelet refractoriness (stubborness) - non response - may be due to excessive consumption or antibody mediated destruction

May indicate selection of HLA or HPA matched donor platelets

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14
Q

What platelet count indicates need for transfusion

A

Platelet count less than 50x10^9/L

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15
Q

What is our commercial factor 8 called

A

Advate

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16
Q

What is our commercial fibrinogen product called

A

Haemocomplettan

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17
Q

When might fibrinogen be given

A

In a mass bleed to stop bleeding

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18
Q

What is our albumin commercial product called

19
Q

What is haemophilia
(3)

A

Inherited bleeding disorders, usually classified as mild, moderate and severe

Either FVIII (Haemophilia A) or FIX (Haemophilia B) deficient

Both X-linked disorders with female carriers and male sufferers

20
Q

What is Haemophilia B also called

A

Christmas disease

21
Q

What lab tests would indicate haemophilia

A

PT normal
APTT prolonged

22
Q

Describe the life of a severe haemophiliac before treatment was made available

A

Usually wheelchair bound by childhood due to bleeds in their joints

Haemorrhaging into tissues and joints fixed their joints in place

23
Q

How did we used to treat Haemophiliacs

A

Used to give them full plasma

24
Q

How do we treat Haemophiliacs now

A

After the discovery of cryoprecipitate we began using factor 8 transfusions

We used to use human-isolated factors (lyophilised)

We now use recombinant products

25
What is cryopercipitate
Discovered accidently when a plasma pack was left in a fridge overnight and it froze The pack contained flakes/precipitates which contains factor 8, VWB and fibrinogen We were able to isolate these to treat haemophiliacs
26
Why can it be difficult to treat haemophiliacs
Can be difficult to separate VWB factor from Factor 8 Can have a very strong antibody response to factor 8 (these antibodies are produced when a person doesn't naturally have this factor, these are called inhibitors)
27
How are our factor 8 concentrates processed today (4)
Lyophilised - suitable for 'home' treatment Heat treated at 60 degrees for 72 hours Solvent-detergent treated to disrupt viral coats of Hep B, C and HIV F VIII gene has been cloned and successfully grown in cell culture media in chinese hamster ovary cells -< recombinant FVIII
28
Give an example of a recombinant factor VIII used in Ireland
Advate Recombinant factor VIII
29
What is von Willebrands Disease (5)
Autosomal inherited lack of VWF Results in mucocutaneous bleeding Platelet function is impaired Prolonged Bleeding time -> often seen when someone gets a tooth removed 3 types from mild to severe
30
Why is VWD similar to Haemophilia A
VWF circulates with FVIII VWF supports factor 8 levels
31
What does VWF do
VWF binds to collagen and to glycoprotein 1b found on platelets Binds to collagen, platelets and factor 8 Its the glue that forms a clot
32
How did we used to treat VWD
Used to give human isolate factor 8 as some human derived factor 8 will have vWF e.g. Humate P Wilate and Cryoprecipitate
33
What is desmopresin and what does it do
A drug that causes an increase in factor 8 and VWF
34
How do we treat VWD today
Use (DDAVP injection) Desmopressin Or Human sourced factor 8 and vWF
35
Write about factor 9 concentrates for Haemophilia B and how they are used in treatment (8)
Often contain factors 2, 7, 9 and 10 Prothromplex Octaplex used for reversal of warfarin overdose Pure factor 9 is available Risk of inhibitors lower for F9 than for F8 Recombinant product available called ALPROLIX or BenefIX
36
What is ALPROLIX
Purified recombinant factor 9
37
Why is it less likely to develop factor 9 antibodies than factor 8 antibodies
Factor 9 is a much smaller molecule
38
What is activated factor 7 and when is it given
Given in severe bleeding in haemophiliacs A or B Factor 7 deficiency is very rare Has Factor 8 inhibitor bypass activity i.e. given to Haemophiliacs A with inhibitors e.g. Novoseven
39
What is cryoprecipitate
Cold insoluble globulin fraction of plasma rich in factor 8, VWF and fibrinogen
40
How is cryoprecipitate made (4)
Fresh frozen plasma is allowed thaw at 4 degrees Excess plasma is siphoned off, leaving ppt and some plasma It is then frozen and stored for use - reconstituted and transfused Has a half life of 12 hours
41
What is a special use of cryoprecipitate
Product can be mixed with thrombin and applied topically to site of bleeding -> Fibrin Sealant/Glue New type of fibrin sealant Used in surgery where there is diffuse bleeding e.g. liver surgery
42
What do we use now instead of cryoprecipitate
Isolated human fibrinogen -> Haemocomplettan P
43
In your own words how is cryoprecipitate made (5)
Plasma frozen at -18 degrees Plasma thawed to 1-6 degrees Plasma centrifuged Supernatant removed (Cryo-supernatant) Remainder in pack is cryo-precipitate