Hemophilia in er
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Transcript of Hemophilia in er
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You are in fast Track ………..
Rashid abuelhassan
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• 26y, M, k/c Hemophilia
• c/o : throat and neck pain, sudden , no trauma
• Vitals : stable
• What you will ask him
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Things to ask?
• Type of hemophilia/factor that he receives
• factor activity
• Hx any inhibitors
• Wt
• Additional bleeding
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• o/e:
• Swelling on the neck
• Auscultation normal
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Management ?
• Factor level
• Assess severity (bleeding type )
• target
• Calculate dose
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• 4y, M, k/c Hemophilia
• c/o : elbow swelling and redness slight hotness
• Vitals : stable
• Will you aspirate ?
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• 18y, M, k/c Hemophilia
• c/o : thigh discomfort while walking and now mild pain, no trauma
• Vitals : stable
• o/e: normal skin , no effusion , Normal ROM
• What will you do ?• GIVE VOLTAREN INJ. &D/C
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• 22y, M, k/c Hemophilia
• c/o : parathesia in leg , no back pain , no trauma
• Vitals : stable
• o/e: normal skin , no effusion , Normal ROM
• Cause can be ?
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Types Type A: Factor VIII ; 85% of cases Type B: Factor IX ; “Christmas disease” X‐linked recessive 30% no family history
clinically indistinguishable
Clinical Presentation Bleeding red flags pain Pain
What will kill your patient? Life‐threatening hematomas: airway, compartment syndrome, retroperitonealBleeds, Retroorbital CNS bleeds
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Workup
Prolonged PTT (may be falsely normal if factor activity is >30%)
Factor activity levels are definitive test
Grading based on factor activity level as a % of normal
Mild: 6‐60% Moderate: 1‐5%
Severe: <1%
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ttt
GI, Neck Throat
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• Factor VIII Required = Wt (kg) x 0.5 x (% Activity Desired – % Intrinsic Activity)
• Factor IX Required = Wt (kg) x 1.0 x (% Activity Desired – % Intrinsic Activity)
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if factor VIII not availabl?
FFP =1U of FVIII per 1mL FFP
Cryoprecipitate= 100U of FVIII per bag (also rich in vWF)
Factor VIII = “V”FVIII raises activity 2% for every U/kg
Factor IX = “1”FIX raises activity 1% for every U/kg
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Other Meds
• Oral and Mucosal Bleeding• tranexamic acid 10 mg/kg IV three times per day for 1 to 7 days
used with caution if hematuria is present because ureteral obstruction due to clot formation has been reported.
• aminocaproic acid 75 to 100 mg/kg q 6 hours for children, and 6 g every 6 hours for adults, given PO or IV
• Mild Hem A: DDAVP: increases FVIII activity x 2‐3 (Onset 30 mins; peak 90 – 100 min)
Dose: 0.3mcg/kg IV/SQ; 150mcg N.S if <50kg; 300mcg if >50kg
• Factor inhibitors, severe haemophiliacs, can cause anaphylaxis during
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Acquired Hemophilia
• inhibitors to clotting factors making treatment more difficult.
• Causes:• Malignancies (CLL, Adenocarcinomas)• Pregnancy or Postpartum State• Autoimmune Disorders (SLE, RA)• Idiopathic• drugs (e.g., penicillin, sulfonamides, ciprofloxacin, phenytoin,clopidogrel
• Clotting Factor Mixing Test• Mix normal plasma with patients serum• Normal = aPTT will normalize• Inhibitor = aPTT remains prolonged
• Ttt:• Low titers of inhibitor (≤5 Bethesda Units) Factor VIII concentrates• High titers of inhibitor (>5 Bethesda Units) factor VIIa conc or (PCCs)
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indications for hospital admission
• treatment requiring multiple factor replacement doses
• necessity for parenteral pain management
• Patients with bleeding in the CNS, neck, pharynx, retropharynx, or retroperitoneum,
• compartment syndrome