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You are in fast Track ……….. Rashid abuelhassan

Hemophilia in er

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Page 1: Hemophilia in er

You are in fast Track ………..

Rashid abuelhassan

Page 2: Hemophilia in er

• 26y, M, k/c Hemophilia

• c/o : throat and neck pain, sudden , no trauma

• Vitals : stable

• What you will ask him

Page 3: Hemophilia in er

Things to ask?

• Type of hemophilia/factor that he receives

• factor activity

• Hx any inhibitors

• Wt

• Additional bleeding

Page 4: Hemophilia in er

• o/e:

• Swelling on the neck

• Auscultation normal

Page 5: Hemophilia in er

Management ?

• Factor level

• Assess severity (bleeding type )

• target

• Calculate dose

Page 6: Hemophilia in er

• 4y, M, k/c Hemophilia

• c/o : elbow swelling and redness slight hotness

• Vitals : stable

• Will you aspirate ?

Page 7: Hemophilia in er

• 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

Page 8: Hemophilia in er

• 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 ?

Page 9: Hemophilia in er

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

Page 10: Hemophilia in er
Page 11: Hemophilia in er

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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Page 13: Hemophilia in er

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

Page 17: Hemophilia in er

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)

Page 18: Hemophilia in er

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