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 Willebrand’s disease
• Glanzmann’s
• Bernard-Soulier
• Storage pool disease – gray platelets
– Acquired
• Drugs
• MDS
• Multiple myeloma – hyperglobulinemia
• Uremia
COAGULATION DISORDERS
• Hemophilias A & B
• Others
• Von Willebrand’s 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.
– Don’t 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 doesn’t 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