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BLEEDING DISORDERS

AN OVERVIEW WITH
EMPHASIS ON EMERGENCIES
CLASSIFICATION
PLATELET DISORDERS AND
VASCULAR COMPONENTS
COAGULATION ABNORMALITIES
OTHER
VASCULAR AND PLATELET
ABNORMALITIES
Vascular - hereditary
Hereditary hemorrhagic telangiectasia
Ehlers-Danlos syndrome
- acquired
senile purpura
Henoch-Schonlein syndrome
Scurvy - Amyloid
Steroid purpura
Acquired Coagulation Disorders
Vitamin K deficiency
Warfarin therapy
Liver disease
DIC
Antibodies against factor VIII (or other
factors)
Massive transfusion
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
Disorders of platelet function
Inherited
Von Willebrands disease
Glanzmanns
Bernard-Soulier
Storage pool disease gray platelets
Acquired
Drugs
MDS
Multiple myeloma hyperglobulinemia
Uremia
COAGULATION DISORDERS
Hemophilias A & B
Others
Von Willebrands disease
Inherited Coagulation Disorders
Factor VIII deficiency may be severe <1%,
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
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
True Emergencies
Hemophilia A or B with bleeding
TTP
DIC
ITP with bleeding
Factor inhibitor with bleeding
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
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
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.
Hemophilia
Diagnosis is based on the Factor level and
the history
Replacement of factor is the main thing
Remember antibodies
Thrombotic Thrombocytopenic
Purpura
Diagnosis is made by finding 3 of 5
features.
Microangiopathic hemolytic anemia
Thrombocytopenic purpura
Fever
Neurologic abnormalities
Renal failure
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.
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
DIC
Diagnsis is ever so important
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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