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HEMATOLOGIC EMERGENCIES

HEMATOLOGIC EMERGENCIES. Jason Mitchell Michael Szava-Kovats Joe Vipond May 17, 2012. HEMOSTASIS. HEMOSTASIS. HEMOSTASIS. HEMOSTASIS. HEMOSTASIS. PTT. PT/INR. CASE. 30 yo M Tooth extraction Consistent oozing Stable DDx? Investigations?. CASE. 63. 135. 140. 111. 130. 7.

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HEMATOLOGIC EMERGENCIES

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  1. HEMATOLOGIC EMERGENCIES Jason Mitchell Michael Szava-Kovats Joe Vipond May 17, 2012

  2. HEMOSTASIS

  3. HEMOSTASIS

  4. HEMOSTASIS

  5. HEMOSTASIS

  6. HEMOSTASIS PTT PT/INR

  7. CASE • 30 yo M • Tooth extraction • Consistent oozing • Stable • DDx? • Investigations?

  8. CASE 63 135 140 111 130 7 4.0 24 INR: 0.9 PTT: 54

  9. von WILLEBRAND DISEASE

  10. von WILLEBRAND DISEASE • THREE TYPES • Type I: Partial quantitative deficiency vWF • AD, Most common (75%) • Type II: Abnormally functioning vWF • AD or AR, Subtypes: IIA, IIB, IIM, IIN • Type III: Total quantitative deficiency vWF • AR, Leads to severe bleeding

  11. von WILLEBRAND DISEASE • INVESTIGATIONS • Plasma vWF:Ag Decreased • Plasma vWF Activity Decreased • FVIII Levels Decreased

  12. von WILLEBRAND DISEASE • MANAGEMENT OPTIONS • Standard measures • DDAVP • vWF Replacement

  13. von WILLEBRAND DISEASE

  14. von WILLEBRAND DISEASE • DESMOPRESSIN (DDAVP/OCTOSTIM) • 0.3 μg/kg IV (MAX 20 μg) SC/IV over 20-30 minutes

  15. von WILLEBRAND DISEASE • vWF REPLACEMENT • Humate P 60 – 80 Units/kg IV

  16. von WILLEBRAND DISEASE • ?r-vWF • Some promising results in animal studies • Currently in phase III human trials.

  17. HEMOSTASIS

  18. CASE • 51 yo M • Fall down 3 stairs, Stable • No LOC, no neuro deficits • No neck pain • C/O R knee pain/swelling • Hemophiliac

  19. HEMOPHILIA • Management priorities?

  20. HEMOPHILIA • HEMOPHILIA A • Factor VIII Deficiency • X-linked recessive • 1:5-10 000 males • HEMOPHILIA B • Factor IX Deficiency • X-linked recessive • 1:25-30 000 males

  21. HEMOPHILIA • Initial Investigations?

  22. HEMOPHILIA 76 105 138 101 115 13 4.1 19 INR: 0.9 PTT: 76

  23. HEMOPHILIA • INVESTIGATIONS • Factor VIII Level Decreased • Factor IX Level Decreased

  24. HEMOPHILIA • MANAGEMENT Depends on: Clotting factor activity level Location of bleed

  25. HEMOPHILIA

  26. HEMOPHILIA • BLEEDING LOCATION LIFE/LIMB THREATENING BLEED MODERATE/MINOR BLEED

  27. HEMOPHILIA • LIFE/LIMB THREATENING BLEED

  28. HEMOPHILIA • MILD/MODERATE BLEED

  29. HEMOPHILIA • MANAGEMENT OPTIONS • Cryoprecipitate • FFP • DDAVP • rFVIII / rFIX • Antifibrinolytics

  30. HEMOPHILIA • CRYOPRECIPITATE • FVIII, vWF, Fibrinogen • 2 bags / 10 kg • Not first line therapy – use if rFVIII not available • FFP • All coagulation factors • Not ideal • Need to double plasma volume level

  31. HEMOPHILIA • DESMOPRESSIN (DDAVP/OCTOSTIM) • 0.3 μg/kg IV (MAX 20 μg) SC/IV over 20-30 minutes

  32. HEMOPHILIA • rFVIII (KOGENATE) • MILD/MODERATE • 30 U/kg • SEVERE • 50 U/kg • rFIX (BeneFIX) • MILD/MODERATE • 50 U/kg > 15 • 70 U/kg < 15 • SEVERE • 120 U/kg > 15 • 160 U/kg < 15

  33. HEMOPHILIA • WHEN IN DOUBT, TREAT AS SEVERE BLEED!

  34. HEMOPHILIA • ANTIFIBRINOLYTICS • Tranexamic Acid • Epsilon Aminocaproic Acid

  35. HEMOPHILIA PLASMINOGEN

  36. HEMOPHILIA • ANTIFIBRINOLYTICS • 25 mg/kg PO TID 1-7 days

  37. HEMOPHILIA • THERAPEUTIC ENDPOINTS • Bleeding cessation • Symptom resolution • Correction of PTT • Raised factor activity level

  38. BACK TO CASE • 51 yo M • Fell down three stairs • Stable • R knee hemarthrosis • HEMOPHILIA A

  39. HEMOPHILIA • What type of bleed?

  40. HEMOPHILIA A

  41. HEMOPHILIA A • Management?

  42. HEMOPHILIA

  43. HEMOPHILIA • Why is the patient not responding?

  44. HEMOPHILIA • INHIBITORS • Formation of anti-factor antibodies (IgG) • 1/3 severe Hemophilia A; 1/50 mild/moderate Hemophelia A • 1/100 Hemophelia B • Can be transient

  45. HEMOPHILIA • INHIBITOR MANAGEMENT • aPCC (FEIBA) • rFVII (NIASTASE) • 90 μg/kg q2h x 3 • or until bleeding stops

  46. HEMOPHILIA A

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