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N935 Pathophysiology > coagulation disorders > Flashcards

coagulation disorders Flashcards

1
Q

single factor deficiencies and anesthesia (treatment/replacement considerations)

A

tx of single factor deficiency depends on severity
for surgery, individual clotting factor levels of 20-25% provide adequate hemostasis
duration of effect for replacement therapy depends on turnover time of each factor
factor levels in different produces vary considerable

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2
Q

products available to treat single factor deficiencies

A

factor concentrates
recombinant factors
FFP
(take it, make it, or give them all)

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3
Q

______ml/kg of FFP is needed to obtain _____% increase in level of any clotting factor

A

15-20ml./kg

20-30% increase

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4
Q

coagulation disorders: hereditary deficiencies

A

hemophilia A
hemophilia B
vWB disease

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5
Q

coagulation DO’s: acquired deficiencies

A 
vitamin K deficiency (from antibiotics, intestinal malabsorption, impaired nutrition)
liver disease (parenchymal disease)
DIC
autoantibodies
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6
Q

congenital factor 8 deficiency: hemophilia A

A

factor 8 gene is a very large gene on the x chromosome
can be inherited or gene mutation
clinical severity is best correlated with factor 8 activity level

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7
Q

severe hemophilia

A

inversion or deletion of major portions
<1% factor VIII activity
diagnosed in childhood (spontaneous hemorrhage into joints, muscles, and vital organs)
requires factor VIII concentrates

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8
Q

mild hemophilia

A

6-30% factor VIII activity

may go undiagnosed until adulthood and undergoing major surgery

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9
Q

diagnosis of hemophilia A or B

A

prolonged aPTT, specific factor testing, and gene testing

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10
Q

anesthesia and hemophilia A

A

hematology consult
factor VIII level should be brought o at least >50% prior to surgery
FFP and cryo can also be used
consider TXA as adjunct

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11
Q

anesthesia and hemophilia A: mild hemophilia A tx

A

DDAVP 30-90 minutes prior to srugery

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12
Q

anesthesia and hemophilia A: moderate to severe hemophilia A

A

factor VIII concentrate
half life of FVIII is approximately 12 hours in adults (short as 6 hours in children)
may require replacement therapy for days to weeks after surgery

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13
Q

congenital F-IX deficiency: hemophilia B

A

similar clinical picture as hemophilia A
also x linked and much less common
factor 9 levels below 1% are associated with severe bleeding
mild disease often not detected until surgery or dental procedure (5-40%)

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14
Q

anesthesia and hemophilia B

A

similar to hemophilia A
hematology consult
replacement therapy
consider txa as adjunct

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15
Q

replacement therapy for hemophilia B

A

recombinant factor 9
purified factor 9
prothrombin complex concentrate (PCC’s) contain II, VII, IX, X (increased risk of thrombotic events, especially in orthopedic surgery)

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16
Q

factor 9 half life

A

18-24 hours. absorbed into collagen and vasculature

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17
Q

von willebrand disease

A

most common congenital bleeding disorder in the world. more prevalent in persons of european descent.
family of disorders caused by quantitative and/or qualitative defect

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18
Q

vWF role

A

mediates platelet adhesion and prolongs factor 8 half life. dual role in hemostasis, affecting both platelet function and coagulation.

  1. platelet adhesion (to vascular site of injury. platelets and G1b receptor)
  2. platelet aggregation (plt GIIb/IIIa receptor to platelet GIIb/IIIa receptor)
  3. carrier molecule for factor VIIII and cofactor for factor 9
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19
Q

v willebrand factor antigen

A

antigenic determinants on vWF measured by immunoassays, usually low in types 1 and 2, virtually absent in type 3

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20
Q

ristocetin cofactor activity (RCo)

A

functional assay of vWF activity based on platelet aggregation with ristocetin. reduced by the same degree as vWF:Ag in types 1 and 3, but to a greater extent in type 2 disease

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21
Q

where is vWF synthesized and stored

A

endothelial cells, platelets

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22
Q

vWF type 1

A

most common in 60-70% of patients
mild to moderate reduction in level of vWF
mild bleeding symptoms, easy bruising and nosebleeds

23
Q

vWF type 2

A

9-30% of patients
qualitative defect of vWF
4 subtypes

24
Q

vWF type 3

A

<1% of patients

nearly undetectable, severe quantitative phenotype

25
vWF platelet pseudo type
defect in platelets GIb receptor
26
what determines approach to treatment in vWF disease
specific type of disease and location and severity of bleeding
27
vWF type 1 first line of treatment
desmopressin test infusion
28
vWF type 1 second line of treatment if first line fails
intermediate or high purity virus activated factor VIII concentrates
29
vWF type 2 or 3 first line of treatment (and what to consider after for 1, 2, or 3)
intermediate or high purity virus activated factor VIII concentrates transfusions of platelets could be required in special circumstances
30
what to monitor for intermediate or high purity virus inactivated factor 8 concentrates
factor VIII level, symptoms, vWF activity, vWF antigen.
31
replacement therapy goals in vWF disease: major surgery
maintain factor VIII level >50% for 1 week | prolonged Treatment in type 3 patients (> 7 days)
32
replacement therapy goals in vWF disease: minorsurgery
maintain factor VIII level >50% for 1-3 days | maintain factor VIII level >20%-30% for an additional 4-7 days
33
replacement therapy goals in vWF disease: dental extraction
single infusion to achieve factor VIII level >50% desmopressin prior to procedure for type 1
34
replacement therapy goals in vWF disease: spontaneous post traumatic bleeding
usually single transfusion of 20-40units/kg (of what?)
35
VIII synonym biologic half life blood product source
anti hemophilic factor 12-15 hours FFP, factor concentrates, cryoprecipitate
36
IX synonym biologic half life blood product source
christmas factor 18-30 FFP, PCC, factor concentrates
37
DIC
thrombin does not stay at the site of vascular injury like normal. instead, thrombin is generated in response to an insulting factor (endotoxins, sepsis, amniotic fluid embolism) leads to intravascular clotting which then disseminates blood clots form throughout the body causing end organ dysfunction coagulation factors deplete and platelets are used up or become dysfunctional fibrinolysis also activated and results in bleeding
38
DIC symptoms
related to widespread clot formation: chest pain, SOB, leg pain, problems speaking or moving patients can present with either clotting, bleeding, or both hemorrhages occur simultaneously from distant sites while there is ongoing thrombosis in microcirculation (IV sites, catheters, drains)
39
causes of DIC
``` sepsis (bacterial, viral, fungal) surrgery trauma cancer (more chronic, insidious onset, often leukemias) pregnancy complications (AFE, HELLP syndrome) snake bites (venom) frostbite burns transfusion reaction ```
40
acute DIC
processes of coagulation and fibrinolysis are dysregulated widespread clotting with resulting bleeding fibrin deposits as thrombosis in the circulation depletion of platelets and clotting factors regardless of the triggering event of FIC, once initiated, the pathophysiology of FIC is similar in all conditions
41
tissue factor
present in many cell surfaces (endothelial, macrophages, monocytes) and tissues (lung. brain, placenta). exposed and relapsed. binds with FVIIa and activates IX and X to form thrombin and fibrin in final common pathway
42
fibrinolysis
creates fibrin degradation products that inhibit platelet aggregation, have antithrombin activity, impair fibrin polymerization. all of which contribute to bleeding
43
paradoxical effect of fibrinolysis in setting of DIC
coagulation inhibitors are also consumed decreased inhibitor levels will permit more clotting increased clotting leads to more clotting thrombocytopenia occurs due to platelet consumption dysfunctional platelets occur due to inflammatory processes
44
thrombin
lots and lots of clotting depletion of platelets and clotting factors fibrin degradation products (FDP) excess and unregulated thrombin generation causes consumption of coagulation factors and increased fibrinolysis which in conjunction with platelet dysfunction can lead to bleeding consumption of anticoagulant proteins with high antifibrinolytic activity and platelet aggregation also induced by thrombin can lead to thrombotic complications
45
blood tests in DIC
lab test abnormalities and clinical presentation of an underlying factor to help diagnose DIC DIC panel (in isolation, they are not helpful) low platelets but also platelet dysfunction low fibrinogen high INR and PT high PTT high D dimer (FDP) variable TEGS and rotems
46
treatment of DIC
recognition treat underlying condition (infection, abx. trauma, resuscitation, remove insult if possible) supportive care (platelets, cryo, fibrinogen concentrate, FFP, heparin, TXA, PCC's)
47
thrombin (DIC)
potent pro inflammatory protein and platelet aggregator | neutralizing thrombin effect is crucial
48
thrombomodulin
binds to thrombin and decreases pro inflammatory response- limited clinical trials
49
direct thrombin inhibitors
no RCTs yet
50
heparin
historically used in DIC with variable outcomes reserved for early or highly prothrombotic states high risk of additive bleeding has been shown to reduce end organ dysfunction often discontinued if/when overt bleeding starts difficult to monitor because PTT is already prolonged due to coagulation factor consumption
51
TXA
in severe trauma, excessive thrombin is generated to rescue the host from excessive bleeding simultaneously, plasmin is generated to breakdown the microvascular clots, but unfortunately is not able to isolate leading to widespread hemorrhage early hyperfibrinolysis may be treated with TXA. consider single upfront bolus during acute period as later the balance is tipped toward thrombosis CRASH-2 trial prospective trials are still needed
52
hyperfibrinolysis management
txa, blood product support procoagulant process is anticipated once bleeding has been controlled, consider antocoagulatn treatment to control ongoing thrombin generation
53
in presenting procoagulant DIC, consider
anticoagulation first and blood product support for bleeding or invasive procedures. LMWH (thrombomodulin trial stages only)
54
blood product support for DIC includes
platelets, then cryo or fibrinogen concentrate, then FFP

N935 Pathophysiology (12 decks)

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