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Heme > Week 3: coagulation disorders > Flashcards

Week 3: coagulation disorders Flashcards

1
Q

What is the molecular dysfunction in Hemophilia A?

Mode of inheritance?

Appearance of PT/PTT test?

A

There is little to no Factor 8.

X Recessive

Prolonged PTT, normal PT, can correct with normal plasma addition

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2
Q

What is the presentation of Hemophilia A?

A

Deep bleeding into joints
Hematomas
Hemarthrosis
Post-circumcision bleeding

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3
Q

How do you treat Hemophilia A?

A

EDUCATION

  1. Desmopressin - increases vWF (which stabilizes and carries factor 8)Only works in patients that can produce SOME factor 8.
  2. Aminocaproic Acid - Inhibits fibrinolysis, allowing the person to clot.
  3. Physical Therapy
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4
Q

What does the platelet count look like in Hemophilia A?

A

Normal

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5
Q

What does the platelet count look like in ALL the hemophilias?

A

Normal. Coag disease. Not platelets.

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6
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand Disease

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7
Q

vWF Disease Mode of Inheritance?

A

AD

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8
Q

What 2 jobs does vWF do and where is it synthesized/stored?

A

Function:
1. Platelet binding to subendothelial collagen

  1. Carries Factor VIII (8)

Synthesized in platelets, MKs, and endothelium.

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9
Q

What do the PT/PTT tests look like in vWF disease?

A

Prolonged PTT because lack of vWF causes lack of Factor 8.

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10
Q

How does a patient with vWF present?

A

Bruising easily

MUCOSAL BLEEDING- nosebleeds (epistaxis) characteristic

Menorrhagia

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11
Q

How do you treat vWF Disease?

A

Desmopressin - increases endothelial release of vWF

Aminocaproic Acid - inhibits Fibrinolysis

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12
Q

What tests do you run to confirm you have vWF?

A

RISTOCETIN TEST - impaired aggregation.

Everything clots with Ristocetin. Except vWF Disease.

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13
Q

How many factors are usually impaired in a hereditary coagulation disorder? What about acquired?

A

Hereditary: ONE

Acquired: Many

  • DIC
  • Drug
  • Liver Disease
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14
Q

What is thrombophilia?

A

An increased tendency to clot

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15
Q

Name the 4 heritable Thrombophilias we talked about.

A

Factov V Leiden

Antithrombin 3 deficiency

Protein C/S deficiency

Prothrombon gene mutation G20210A

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16
Q

What is the most common inherited Thrombophilia?

A

Factor V Leiden mutation

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17
Q

What is the Pathology of Factor V Leiden mutation?

A

Factor V has an Arg506Glu mutation that makes it resistant to degradation by Protein C.

18
Q

How do you test for Factor V Leiden mutations?

A

Add Protein C and observe PT/PTT. If they are both NORMAL, then Factor V is resistant.

(+) test for Factor V Leiden mutation

Then PCR Screen for the mutation

19
Q

When do you treat Factor V Leiden mutation?

A

When a thrombus occurs, or when there is significant risk of one. (long flights)

If there are no thrombi present, and no history of them, DONT TREAT.

20
Q

How do you test for an Antithrombin III deficiency?

A

Inject Heparin and look at the PTT. There should be no change in the PTT because Heparin has nothing to bind to.

21
Q

How do you treat Antithrombin III deficiency?

A

Antithrombin replacement

22
Q

How does deficiency in Proteins C and S present? (Think Warfarin)

A

Skin necrosis, thrombosis

Depletion of Proteins C and S remove inhibition from the coagulation cascade, letting it go crazy.

23
Q

What happens if you have a Prothrombin Gene Mutation G20120A?

A

There is sustained generation of thrombin in the blood plasma.

Increased thrombosis

Decreased Fibrinolysis

24
Q

A patient presents with spontaneous skin purport, mucosal bleeding, and excessive bleeding when wounded. How do you know if this is a Thrombocytopenia or vWF disease?

A

RISTOCETIN TEST

Won’t clot if it’s vWF

25
Q

A 30 year old woman comes in with headaches, dizziness, renal insufficiency, and fever. What does she most likely have?

A

TTP TRIAD:

Thrombotic Thrombocytopenic Purpura

26
Q

What is the molecular abnormality in TTP?

A

2 Types:

Congenital: Absence/Defective ADAMTS13

Acuired - IgG antibody to ADAMTS13

27
Q

What is ADAMTS13?

A

The metalloprotease responsible for cleaving vWF to the correct size so it can be degraded once clot is formed.

If vWF isn’t cleaved, platelets keep piling on the clot.

28
Q

What will you see on the peripheral smear of a person with TTP?

A

Schistocytes due to Microangiopathic Hemolytic Anemia

29
Q

How do you treat TTP?

A

Plasmaphoresis

30
Q

A young woman comes in with symptoms of thrombocytopenia but no neutropenia or anemia. She seems otherwise healthy, except for the bruising and petichiae. What does she most likely have?

A

ITP - Idiopathic Thrombocytopenic Purpura

31
Q

What is the pathogenesis of ITP?

A

Idipathic THrombocytopenic Purpura

IgG antibodies against GP1b or GPIIb/IIIa receptors

Antibodies tag the platelets and they are removed by the spleen

32
Q

What is the precursor to ITP in kids?

A

URI, mono, chicken pox. Some kind of infection.

MOST COMMON BLEEDING DISORDER IN KIDS

33
Q

How do you treat a kid with ITP>

A

You don’t. It’ll go away.

34
Q

How do you treat an adult with ITP?

A

Corticosteroids

IVIG (give the spleen something else to munch on)

TPO agonists

Splenectomy (last resort)

35
Q

How do you treat DIC?

A

Treat the underlying cause. You can’t have JUST DIC. It has to be as a result of another condition.

36
Q

What is DIC?

A

constitutive activation of the coagulation cascade.

EXCESS THROMBIN PRODUCTION –> abnormal fibrin deposition –> clots –> Microangiopathic Hemolytic Anemia

37
Q

What lab tests confirm DIC?

A

Low platelets (thrombocytopenia)
LOW FIBRINOGEN
Low, exhausted clotting factors
HIGH D-DIMER (fibrinolysis product)

38
Q

What does DIC look like?

A

Petechiae, Purpura, Peripheral Gangrene due to microvasculature ischemia

39
Q

What do you look for in Hereditary Hemorrhagic Telangiectasia?

A

Bleeding on the tongue (petechiae)

Recurrent GI bleeding –> Iron deficiency

40
Q

Senile Purpura looks like what?

A

Senile Purpura

Heme (9 decks)

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