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IMHA, ITP, Evans Syndrome: The Body Attacking Itself

IMHA, ITP, Evans Syndrome: The Body Attacking Itself. Jennifer M. Pearson, DVM, MS Diplomate ACVIM (SAIM) Peak Veterinary Specialsists and Emergency October 16, 2013. Doctors. Internal Medicine Dr. Jennifer M. Pearson Dr. Sacha Mace Surgery Dr. Gerardo Belandria

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IMHA, ITP, Evans Syndrome: The Body Attacking Itself

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  1. IMHA, ITP, Evans Syndrome: The Body Attacking Itself Jennifer M. Pearson, DVM, MS Diplomate ACVIM (SAIM) Peak Veterinary Specialsists and Emergency October 16, 2013

  2. Doctors • Internal Medicine • Dr. Jennifer M. Pearson • Dr. Sacha Mace • Surgery • Dr. Gerardo Belandria • Dr. Preson Stubbs • Dentistry • Dr. Clarence Sitzman • Emergency • Dr. Laura Halpin • Dr. Elizabeth Fogel • Dr. Nicole Vumbaco • Dr. Renea Keller • Dr. Anna Rothman

  3. Technicians • Internal Medicine • Mindy Lott • Kelsey Schneider • Keeley Cook • Cassidy Martin • Susan Faulkner • Shelly Cornutt • Swing/Emergency • Alyssa Ortiz • ChelseeJacobs • Vihaney Graves • Samantha Loeber • Eric Howard • James Snedaker • Andrea Ferguson • Jami Warino

  4. Other support Staff • Front Desk • Jane Snyder • Monika Scherer David Johnston • Hospital Administrator Jen Morris

  5. Technicians Week!!!!!

  6. Agenda • Immune system • Physiology • Immune mediated disease • Pathophysiology • Immune mediated diseases • IMHA • ITP • Evans syndrome • Others • Treatment • Prognosis

  7. Immune System • It’s complicated!!!!

  8. Immune system • Main function • To protect against disease (bacteria, viruses, parasites, etc) • Must differentiate the diseases from healthy tissue • Recognition of self

  9. Hypersensitivity • Immune response resulting in damage to tissues • Type I • - Immediate or anaphylactic reaction (allergies) • - IgE • - mild discomfort or death • Type II • - Antigens bind to antibodies on the patients own cells • - IgM and IgG • - Marks the cell for destruction

  10. Hypersensitivity (cont.) • Type III • - occurs in certain tissues • - Basically type II hypersensitivity with the activation of compliment • - very destructive to cells • Type IV • - aka delayed hypersensitivity • - takes between 2-3 days to develop • - poison ivy

  11. When the immune system goes wrong • 1. Immunodeficiency: lack of normal immunity • More prone to infections • 2. Autoimmune disorders • - hyperactive immune system • - body attacks normal tissues as if they are foreign • 3. Cancer

  12. Causes of Autoimmune Disorders:Primary and Secondary • Primary • Abnormal stimulation to the immune system • No Recognition of self • No underlying cause • Secondary • Underlying cause • Medications • Penicillin antibiotics • Sulfa antibiotics • Cephalosporin antibiotics • Vaccinations • Neoplasia • Hemangiosarcoma • Lymphoma • Infections • Bacterial, viral • Rickettsial

  13. Initial Diagnostics • CBC w/ reticulocyte count • Chemistry profile • Urinalysis • Thoracic radiographs • Abdominal radiographs • Abdominal ultrasound

  14. Specific immunologic diagnostics • Saline Autoagglutination • Coomb’s test • Platelet Autoantiobody • Antinuclear antibody • Lupus erythematosis cell detection • Rheumatoid factor

  15. Signalment for Autoimmune Disorders • No sex predilection • Age • Used to be young dogs • Now no age predilection • Breeds • Cocker Spaniels • Poodles • Any breed, any age, any time any place!!!!

  16. IMHA

  17. Who hates IMHA???? • Resistant • Difficult to treat • Seeing more intravascular hemolysis • Standard Poodles??????

  18. It’s A War • Highly inflammatory process • Ag/Ab complexes can form in different organs • Bone marrow is struggling to keep up • Organ failure • DIC

  19. IMHA Clinical Signs • Weakness • Lethargy • Fever • Pallor • Icterus • Tachypnea • Exercise intolerance • Tachycardia • Anorexia • Vomiting

  20. Blood work abnormalities • CBC • Elevated WBC • Anemia • Regenerative • Elevated MCV • Chemistry profile • Elevated Total bilirubin • Possibly elevated liver enzymes • Azotemia • Urinalysis • Hemoglobinuria • Hemoglobinemia • Elevated USG • Casts??

  21. Anemia • Classification • Regenerative • Nonregenerative

  22. Anemia • Normocytic normochromic • Bone marrow issues • Blood loss • Microcytic hypochromic • Iron deficiency anemia • GI hemorrhage • Macrocytic and normochromic • Hemolytic anemia • Hemorrhage

  23. Anemia from GI Hemorrhage • Can be regenerative or nonregenerative • Microcytic /hypochromic

  24. Regenerative Anemia or Nonregenerative??? • Can initially look nonregenerative • Takes the bone marrow 3-5 days to respond after acute hemorrhage or hemolysis

  25. Hemolysis vs. Hemorrhage • Hemolysis • TP normal • Albumin normal • Icterus • Spherocytes • Hemorrhage • Evidence of blood loss • Low albumin • Low total protein • No icterus

  26. IMHA • Extravascular hemolysis • Intravascular hemolysis

  27. Extravascular hemolysis • IgG and possible IgM • Spleen is main cause for hemolysis • Spherocytes • Possible autoagglutination • Icterus

  28. Spherocyte Formation

  29. Intravascular Hemolysis • IgG and IgM • Activate complement • Very severe • Prognosis poor

  30. Intravascular Hemolysis • Severe icterus • Hemoglobinemia • Hemoglobinuria • Severe autoagglutionation • Organ failure • DIC

  31. Intravascular Hemolysis Hemoglobinuria: Portwineurine!

  32. ITP Antibodies attached to platelets Destruction once released from bone marrow

  33. Clinical Signs of ITP • Bleeding • Bleeding • Bleeding • Epistaxis • Scleral hemorrhage • Hematuria • Melena/Hematochezia • Petechia • Ecchymosis • Bleeding from gums • Lethargy • Weakness • Fever • Anorexia • Vomiting • W/ or w/o blood

  34. Clinical Signs (cont.) • Fever • Lethargy • Petechiae • Other evidence of hemorrhage • Epistaxis • Hematuria • Bleeding from gums • Hematochezia • Hemoptosis • Scleral or ocular hemorrhage • Other

  35. Thrombocytopenia • Consumption • Destruction • Loss (usually GI) • Lack of production

  36. Blood Work Abnormalities • CBC • Elevated WBC • Monocytosis?? • Thrombocytopenia • +/- Anemia • Secondary to hemorrhage • Chemistry profile • Normal • Possible azotemia • Dehydration

  37. How do we determine reason? • Platelet autoantibodies • Bone marrow aspirate • Response to treatment

  38. Megakaryocyte

  39. Evans Syndrome IMHA ITP Fairly common More difficult to treat?

  40. Goals of Treatment • Treat underlying cause • Suppress the Immune system • Immediate • Long term • Support

  41. Treat underlying cause • Must identify underlying cause first • Neoplasia • Bacterial infection • Rickettsial infection • Drug related • Vaccinations

  42. Immunosuppressive drugs • Prednisone • 2-4mg/kg/day po bid • Cyclosporine • 5-10mg/kg po bid • Azathioprine • 1-2mg/kg /day • Cyclophosphamide • 200mg/m2 IV • Leflunomide • Methotrexate • Danazol • Gold salts

  43. Other treatment • Splenectomy • Intravenous gamma globulin • Blood transfusion • Platelet rich plasma

  44. Anticoagulant Tx • Low dose aspirin • 0.5mg/kg po bid • Clopidegrel • ¼ tab for a small dog q 24hrs • Extrapolate from there • Coumadin????

  45. Complications • Lack of response • Life threatening hemorrhage • DIC • Organ failure • Pulmonary thromboemboli

  46. Long Term Considerations • Relapses common • Long term or indefinite treatment • Avoid stimulation to immune system • No further vaccinations • Use certain medications with caution • Monitor careful in the presence of other disease

  47. Prognosis • Variable • Quick response • Delayed response • Death • Relapses

  48. OPEN 24/7 FOR EMERGENCY!!!!

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