Clotting Disorders

last authored: Oct 2009, Dave LaPierre

 

Introduction

 

 

 

 

The Case of...

a simple case introducing clincial presentation and calling for a differential diagnosis. To get students thinking.

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Causes and Risk Factors

 

Clotting disorders are characterized by bleeding into joints and muscles, as well as forming large, spreading ecchymoses and hematomas.

 

Platelet disorders are characterized by peteciae, purpura, bruising, mucocutaneous bleeding (epstaxis, ginigival bleeding), mennhoragia, and bleeding from superficial cuts.

 

In the eldery, do a malignancy workup.

 

target

 

condition

 

mechanism

 

coagulation cascade

hemophilia A

hemophilia B

von Willebrand disease

hemorrhagic disease of the newborn

factor VIII deficiency

factor IX deficiency

abnormal vWF

vitamin K deficiency

platelets

idiopathic thrombocytopenic purpura (ITP), infection, drugs

DIC, hemangioma, hypersplenism

vonWillebrand disease, ASA, uremia

marrow infiltration, leukemias, drugs

increased destruction

increased consumption

dysfunction

decreased production

blood vessels

Henoch-Schonlein purpura

vasculitis

 

 

HELLP stands for hemolysis, elevevated liver enzymes, low platelets

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Pathophysiology

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Signs and Symptoms

  • history
  • physical exam

History

Can be platelets or coagulation cascade.

 

Bruises bigger than a size of a toonie.

Locations not likely to be trauma.

Nosebleeds (epistaxis): duration, oozing from both nostrils.

Hematemesis

 

Petechiae can be normal on chest and neck following vigorous coughing, vomiting, etc.

It is, however, a red flag, suggestive of platelet dysfunction.

Physical Exam

 

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Assessing Bleeding

  • prolonged INR
  • low platelets
  • prolonged PTT
  • normal values

Prolonged INR

 

50:50 correction suggests factor deficiency.

VII deficiency - genetic

 

X, IX, VII, II

  • warfarin
  • vitamin K deficiency

Low Platelets

 

Increased INR, PTT

 

Suggests DIC

 

Prolonged PTT

 

50:50 correctible

VIII, IX

sex-linked, hemophilia A and B

PTT 60-80s

 

 

XI, XII

autosomal dominant

 

VWB

normal platelet numbers, but increased PFA (platelet function analysis)

 

50:50 - non-correctible

inhibitory antibody

  • someone with hemophilia who has been getting VIII replacement
  • elderly (cause unknown; difficult to treat)
  • lymphoma
  • pregnancy (transient)

Treatment

  • emergent: exogenous activated F VII
  • imunosuppressants (cytoxan, prednisone)
  • IVi

 

heparin (commonly from line flushing)

  • identify with thrombin time (TT) or hepabsorb

APLA

 

 

Normal Values

 

Normal platelets, INR, PTT

Suggests dysfunctional platelets

  • ASA, clopidogrel
  • uremia (prevents aggregation)

PFA (platelet function analysis) will show dysregulation

 

 

 

Treatments

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Consequences and Course

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The Case of...

Case #2 - a small story wrapping it all up and asking about esp management.

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Additional Resources

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Topic Development

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