Clotting Cascade Flashcards

1
Q

What factors make up the extrinsic clot pathway?

A

VII

X

II (thrombin)

fibrinogen–> fibrin

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2
Q

What factors are invovled in the Intrinsic pathway?

A

PK to XII

XI

IX

X (and VIII)

II (and V)

fibrinogen–> fibrin

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3
Q

What is the role of Vit K in the clotting cascade?

A

(glutamate—>carboxylated–> glutamic acid) and need it to make

X, IX, VII, and II

PC and S

diSCo in 1972

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4
Q

Where do the two pathways meet?

A

at factor X

intrinsic has IX activate X

Extrinxic has VII activate X

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5
Q

What is the partial thromboblastin time a measure of? (aPTT)

What is the Pro-Thrombin time measuring?

A

aPTT measure of the intrinsic pathway

PT measures the extrinsic pathway

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6
Q

High Molecular Weight Kininogen deficiency Prekallikrein deficiency

  • *FXII** deficiency
  • *FXI** deficiency
  • *FIX** deficiency
  • *FVIII** deficiency

Passovoy Deficiency

Lupus anticoagulant

All cause

A

Isolated prolongued aPTT

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7
Q

What causes isolated PT pronlongation?

A

Factor FVII defiency

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8
Q

What can cause both a prolongued aPTT and PT

A

FX deficiency
FV deficiency
FII deficiency

Fibrinogen deficiency

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9
Q

What can cause a long TCT

A

Dys/hyper/hypo/A fibrinogenima

FDPs, immunoglobins, heparin

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10
Q

What disorders will we see bleeding but they have normal screens

A

Mild factor deficiency

Thrombocytopenia

Thrombocytopathia
Vascular abnormality (scurvy)

FXIII deficiency

Antiplasmin deficiency

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11
Q

Post op 5 yo boy tonsils out, can’t get them to stop bleeding, no sig medical, family hx and ROS no history of bleeding or bruising and normal PE

WBC= 10.1 (nml 5.0-14.0)
Hemoglobin = 13.0 g/dL (nml 11.5-15.5)

Platelet count = 210 (nml 150-450)

Prothrombin time= 11 (nml 11-13.5)

INR= 1.0
Activated partial thromboplastin time (PTT)= 41sec (nml 25-32)

next step?

A

get a Partial thromboplastin time with 1:1 mixing

if it corrects then we know its a cloting factor deficiency

if it doesn’t, we know its some sort of inhibition

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12
Q

How do you manage a pt that has bleeding in the throat that needs to be stopped but you are unsure of the cause of the bleed?

A

– Assessment of the patient’s airway with intubation if required

– IV fluids

– Type and cross for blood transfusion

– Surgical hemostasis

– Further lab studies

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13
Q

You have a pt with prolonged aPTT of 42 secs (normal is 25-32) which corrected after a 1:1 mixing

What could be cause?

A

This is factor deficency in the INTRINSIC path

could be : VIII, IX, XI, XII as most likely

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14
Q

What is hemophelia A and how is it inherited?

What about hemophelia B?

A

Hemophilia is an inherited bleeding disorder in which there is a deficiency or lack of factor VIII (hemophilia A) or factor IX (hemophilia B)

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15
Q

What is the inheritance pattern for hemophelia?

how does it affect males and females?

What is the severity level?

A
  • Hemophilia A and B are X-linked recessive disorders
  • Hemophilia is typically expressed in males and carried by females
  • Severity level is consistent between family members
  • ~30 % of cases of hemophilia are new mutations
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16
Q

What are common complications in pts with hemophelia?

A

Joint bleeds and hemarthrosis or deep tissue bleeds (non mucocutaneous)

need to use rFVIII prior to procedures

17
Q

What is a concern when using recomb FVIII?

A

development of inhibitor (antibody) against VIII

you would see that by performing a 1:1 mix study and the PTT would NOT correct anymore

18
Q

Describe process of developing antibodies to fVIII or fIX

A

Polyclonal high affinity IgG Ab that neutralize the procoagulant function of fVIII or fIX

Factor replacement therapy becomes ineffective unless antibody is removed

Most serious complication of factor replacement therapy

Presents major economic and clinical challenges.

19
Q

• Formation of a fibrin clot (secondary hemostasis) requires initiation of the coagulation cascade by ____ for intrisic and ____through the extrinsic pathway

A

tissue factor (TF)

factor VIIa

20
Q

Fibrin clot is propagated by the

A

instrinsic pathway

21
Q

Deficiencies in _______ result in prolongation of aPTT with normal PT

A

factors VIII, IX, and XI

22
Q

Correction of a coagulation study (aPTT or PT) on a 1:1 mix is diagnostic of

A

a deficiency of clotting factor

23
Q

Hemophilia is an ____ disorder

• Bleeding pattern in hemophilia is characterized by :

A

X-linked

joint, muscle and soft tissue bleeding