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BLEEDING DISORDERS

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Title: BLEEDING DISORDERS


1
BLEEDING DISORDERS
  • AN OVERVIEW WITH
  • EMPHASIS ON EMERGENCIES

2
CLASSIFICATION
  • PLATELET DISORDERS AND VASCULAR COMPONENTS
  • COAGULATION ABNORMALITIES
  • OTHER

3
VASCULAR AND PLATELET ABNORMALITIES
  • Vascular - hereditary
  • Hereditary hemorrhagic telangiectasia
  • Ehlers-Danlos syndrome
  • - acquired
  • senile purpura
  • Henoch-Schonlein syndrome
  • Scurvy - Amyloid
  • Steroid purpura

4
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5
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6
Acquired Coagulation Disorders
  • Vitamin K deficiency
  • Warfarin therapy
  • Liver disease
  • DIC
  • Antibodies against factor VIII (or other factors)
  • Massive transfusion

7
  • Thrombocytopenia
  • False reading
  • Clumping of platelets
  • Platelet satelitism
  • Margination in congestive splenomegaly
  • True thrombocytopenia
  • Failure to produce platelets
  • Acquired - drugs - alcohol
  • Inherited TAR, May-Hegglin. Wiscott-Aldrich
  • Platelet destruction
  • Immune
  • TTP
  • DIC

8
  • Disorders of platelet function
  • Inherited
  • Von Willebrands disease
  • Glanzmanns
  • Bernard-Soulier
  • Storage pool disease gray platelets
  • Acquired
  • Drugs
  • MDS
  • Multiple myeloma hyperglobulinemia
  • Uremia

9
COAGULATION DISORDERS
  • Hemophilias A B
  • Others
  • Von Willebrands disease

10
Inherited Coagulation Disorders
  • Factor VIII deficiency may be severe lt1,
    moderate 1-5, or mild 5-20 activity
  • After repeated doses of Factor VIII patients may
    develop and antibody against the factor
  • Treatment then requires bypassing the factor to
    effect hemostasis with FEIBA or Factor VIIa
  • Rarely the low concentration of antibody can be
    taken up by larger amounts of factor VIII

11
  • Other inherited factor deficiencies
  • Factor XI mostly in Ashkenazi Jews
  • Bleeding is usually mild except after surgery or
    significant injury
  • XI concentrate may be available or give plasma
  • Factor XIII deficiency is rare and is similar to
    XI deficiency in clinical presentation
  • Factor VII and X deficiencies are seen and are
    variable in severity from case to case

12
True Emergencies
  • Hemophilia A or B with bleeding
  • TTP
  • DIC
  • ITP with bleeding
  • Factor inhibitor with bleeding

13
True Emergencies
  • Remember that bleeding can occur because of
    injury with no bleeding diathesis!
  • Hemophilia A or B with bleeding
  • TTP
  • DIC
  • ITP with bleeding
  • Factor inhibitor with bleeding

14
Management of the Emergency
  • Be sure you have a proper diagnosis.
  • Remember the things you must not do, if there are
    any.
  • Be sure that the institution has the capacity to
    deal with the problem.
  • Available medications
  • Available procedures and equipment

15
Injured or Postoperative Patients
  • With no history of bleeding in the past (or no
    history at all)
  • Do the diagnostic things PT/PTT, Plt and PFT
  • If above are normal think of an open vessel.

16
Hemophilia
  • Diagnosis is based on the Factor level and the
    history
  • Replacement of factor is the main thing
  • Remember antibodies

17
Thrombotic Thrombocytopenic Purpura
  • Diagnosis is made by finding 3 of 5 features.
  • Microangiopathic hemolytic anemia
  • Thrombocytopenic purpura
  • Fever
  • Neurologic abnormalities
  • Renal failure

18
TTP
  • Things to avoid.
  • Dont waste time. This is a serious, rapidly
    progressive and fatal illness.
  • Transfusion of blood cells, especially platelets,
    can accelerate the disease.

19
TTP Treatment
  • Plasma infusion in large amounts. This almost
    always means plasmapheresis, using plasma to
    replace volume for volume.
  • Other treatment with pheresis includes
  • ASA and persantine
  • Prednisone

20
DIC
  • Diagnsis is ever so important

21
TTP Warning!
  • Do not try to take care of TTP in a hospital that
    doesnt have pheresis and a blood bank able to
    handle the demands.
  • Immediate transfer to a hospital that has these
    capacities is in order.
  • Before transfer, you might consider giving 2 3
    units of FFP
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