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