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Coagulation Cases. Mike Webb Division of Clinical Hameatology 1 March 2010. So what does it take to make a healthy blood clot?  Platelets The blood vessels themselves Clotting factors. Good vs bad. Healthy blood clot:  At the site of a broken blood vessel, the point of injury.

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Coagulation cases
Coagulation Cases

Mike Webb

Division of Clinical Hameatology

1 March 2010



Good vs bad
Good vs bad

  • Healthy blood clot:

    •  At the site of a broken blood vessel, the point of injury.

    •  A healthy clot stays put and does not cause a runaway clotting episode.

    • A healthy clot knows when it's time to dissolve.

  • Pathological clot:

    • It causes vascular narrowing or outright occlusion.

    • Often inappropriately forms

    • It may embolize.


Evaluation of haemostasis
Evaluation of haemostasis

  • History:

    • Site

    • Recent onset vs. life-long pattern.

    • Alcohol consumption.

    • Use of NSAIDs.

    • Males and females equally affected?

    • Grandparents?

    • Does it skip a generation?

    • Bad arthritis of the ankles or knees in a male grandparent may indicate a clotting factor problem (IX or VIII).




Laboratory evaluation of hemostasis1
Laboratory Evaluation of Hemostasis

  • FBC and smear

  • PT/PTT

  • Plt function

  • Specialized tests

  • Quality of the blood specimen

    • Volume

    • Clot

    • Hemolyzed




Simple
Simple!!

PTT = XII, XI, IX, VIII

PT = VII

Common = I,II,V,X


PT = VII

Common = I,II,V,X


Prolonged pt or ptt
Prolonged PT or PTT

  • Not enough of a single or combination of factors.

  • Does NOT tell you why.


Why?

  • Deficency

    Congenital

    Factor VIII, IX

    Acquired

    Liver diease

    Warfarin

  • Inhibitor

    Lupus

    Heparin

    Acquired Ab vs VIII, IX


Defn vs inhibitor mixing studies
Defn vs Inhibitor:Mixing studies


Which factor
Which factor?

PT = VII

PTT = XII, XI, IX, VIII

Common = I,II,V,X


Tommy active four year old
Tommy active four-year old

  • Scalp wound that won't heal.

  • Bled more than expected from his circumcision.

  • The mother's father had terrible arthritis of his knees and ankles:

    • He was wheelchair-bound by the age of 45.

    • Died of a 'bleeding ulcer' at age 51.

  • Physical exam – normal except for scalp

    wound.


  • Lab results

  • FBC - normal

  • PT = 11 sec (ref = 10 - 15sec)

  • PTT = 54 sec (ref = 25-36sec)

PT = VII

PTT = XII, XI, IX, VIII

Common = I,II,V,X


What do we know
What do we know?

  • Diagnosis?

  • Special investigations?


Hemophilia
Hemophilia

  • Congenital defn of either

    • VIII = A

    • IX = B

  • A usually more common and severe

  • Both VIII and IX deficiencies are sex-linked recessive traits.



Forty eight year old man with ease of bruising
Forty-eight year-old man with ease of bruising.

  • Mr. BA complains of ease of bruising

  • He was fine until about 8 months ago.

  • FBC normal with round macrocytes.

  • PT is 18 sec (N=10-15)

  • aPTT is 48 sec (N= 25 - 36)


Pt ptt prolonged
PT/PTT prolonged

PTT = XII, XI, IX, VIII

PT = VII

Common = I,II,V,X


Both pt and ptt prolonged
Both PT and PTT prolonged??

  • Diagnosis

  • Special Investigations



Tiffany is a 15 year old girl with a rash
Tiffany is a 15 year-old girl with a rash

Her mom says she had a cold about 2 weeks ago.

  • She was fairly sick with upper respiratory symptoms for about 3 days.

  • She improved quickly, and returned to school.

  • Now she has a 'rash' from navel down both legs.

  • Today, she is otherwise healthy and is afebrile.

  • Tiffany has a little sister who is well.

  • No one else in the family is sick.

  • Exam NAD but for rash



    • Lab tests

      • PT = 11 seconds (ref = 11.4 seconds)

      • aPTT = 29 seconds (ref = 23 - 32 seconds)

    • FBC

      • WCC – 8

      • Hgb – 13

      • Plt – 9 (N = 150-450)


    Viral exanthem vs petechiae
    Viral exanthem vs. petechiae?

    • Tiffany's spots are regional, not all over her body.

    • In fact, they seem gravity dependent.

    • They vary slightly in color from red to blue-brown.


    Mrs kl 28 yr old teacher complains of ease of bruising
    Mrs. KL 28 yr old teacher complains of ease of bruising

    • Bleeds excessively with dental procedures

    • She also has menorrhagia

    • She claims if she takes just one aspirin, she will bruise for a week.

    • Her family history is significant for bleeding problems.

      • Some of her relatives, both male and female, have a bleeding tendency.

      • She's very concerned because an aunt supposedly died in childbirth from a hemorrhage.


    Lab results
    Lab results

    • PT = 11.2 seconds (ref = 10-15)

    • aPTT = 42 seconds (ref = 25-36)

    • FBC - normal

    • Factor VIII (enzymatic)

      • 45% (ref 50-150%)


    Von willebrand disease
    von Willebrand disease

    • VWD - most common inherited bleeding disorder. (1% of population)

    • Abnormality in quantity or quality of vWF

      • Binds platelets to endothelium

      • Protects Factor VIII from degredation


    vWD

    • Symptoms are very mild or lack of recognition (heavy menstrual bleeding)

    • Bleeding symptoms:

      • Easy bruising

      • Skin bleeding

      • Prolonged bleeding from mucosal surfaces

    • May mimic hemophilia

    • Ingestion of aspirin or NSAIDS may precipitate bleeding


    vWD

    • Lab tests:

    • Normal FBC

    • Normal PT

    • Normal or prolonged PTT

    • Specialized tests

      • von Willebrand factor

      • vWF Antigen: decreased

      • Ristocetin cofactor activity

      • Platelet aggregation to Ristocetin


    50 yr old man for hip replacement
    50 yr old man for hip replacement

    • On heparin pre-op for DVT prophylaxis

    • Malena stool and epigastic pain

    • FBC – normal

    • PT – 18 (N-10-15sec)

    • PTT >180 (N- 25-36sec)



    Treatment
    Treatment?

    • Stop heparin!

    • Antidote – protamine sulphate


    Tommy late bleeder
    Tommy Late bleeder

    • Known with haemophilia A

    • 28 yrs old presents with painful knee

    • No improvement to factor replacement


    • Mixing studies

    • PT 14 (10-15sec)

    • PTT 105 to 104 (25-36sec)

    • Factor VII = 2%

    • NOW WHAT???


    Late bleeder
    Late bleeder

    PT = VII

    PTT = XII, XI, IX, VIII

    Common = I,II,V,X


    Normal fbc pt ptt
    Normal FBC,PT/PTT

    • vWD

    • Plt dysfunction

    • Vasculitis


    Prolonged ptt normal pt
    Prolonged PTT (normal PT)

    • Intrinsic pathway abn:

      • Hemophilia

      • vWD

      • Heparin

      • Lupus anticoag

      • Auired inhibitors


    Prolonged pt normal ptt
    Prolonged PT (normal PTT)

    • Extrinsic pathway:

      • Vit K defn

      • Liver disease


    Prolonged pt and ptt
    Prolonged PT and PTT

    • Combined defn

    • Warfarin

    • Heparin

    • DIC

    • Liver disease

    • Deficency / inhibitor of common pathway


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