BLEEDING DISORDERS

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BLEEDING DISORDERS

• AN OVERVIEW WITH
• EMPHASIS ON EMERGENCIES

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CLASSIFICATION

• PLATELET DISORDERS AND VASCULAR COMPONENTS
• COAGULATION ABNORMALITIES
• OTHER

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VASCULAR AND PLATELET ABNORMALITIES

• Vascular - hereditary
• Hereditary hemorrhagic telangiectasia
• Ehlers-Danlos syndrome
• - acquired
• senile purpura
• Henoch-Schonlein syndrome
• Scurvy - Amyloid
• Steroid purpura

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Acquired Coagulation Disorders

• Vitamin K deficiency
• Warfarin therapy
• Liver disease
• DIC
• Antibodies against factor VIII (or other factors)
• Massive transfusion

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• 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

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

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COAGULATION DISORDERS

• Hemophilias A B
• Others
• Von Willebrands disease

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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

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• 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

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True Emergencies

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

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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

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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

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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.

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Hemophilia

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

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Thrombotic Thrombocytopenic Purpura

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

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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.

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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

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DIC

• Diagnsis is ever so important

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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