The most expensive sore throat I have ever seen'''

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The most expensive sore throat I have ever seen...
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Clinical presentation History

• 82-year old man
• Cardiac heart failure (hypertensive
  cardiomyopathy)
• Chronic atrial fibrillation
• Usual medications
• Aprovel (ARA2)
• Digoxine
• Lexomil (benzodiazepin)
• Furosemide 40 (diuretics)
• DiffuK (potassium)
• Previscan (Vitamin K antagonist)

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Clinical presentation History

• 15/07/2006 During the night, apparition of a neck
  pain with dysphagia
• 16/07/2006 Family physician Upper respiratory
  tract infectious disease? Augmentin
  (amoxicillin clavulanic acid)
• 19/07/2006 Presented to the ER because of
  dysphagia and neck pain. No change in the
  medication
• 20/07/2006 Apparition of an haematoma of the
  neck. Back to the ER. No other abnormality in
  physical examination

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Clinical presentation Biology
20/07/2006 Hemoglobin 13.8 g/dL Platelet
count 361,000 G/L PT 13, INR 9.9 aPTT gt 180
sec
Vitamin K1 10 mg
21/07/2006 INR 1.35 22/07/2006 INR
1.27 24/07/2006 INR 1.25, aPTT 80
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Clinical presentation Evolution

• 21-27/07/2006
• New haematomas legs, arms, feet
• 24/07 Hb 11.8
• 25/07 Hb 10.9
• 27/07 Hb 8.2, two red cells pack transfusion

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Clinical presentation Evolution

• 28/08/2006 Transfer to internal medicine
  department
• Diffuse subcuatneous haematomas
• No personal or family history of bleeding
• PT 73, aPTT 34/83
• Fibrinogen 4.86 g/L
• D-Dimer 1.19 microg/mL (Nlt0.5)
• Mixing study no aPTT correction
• Rosner H2 27 (Nlt15) circulant anticoagulant
• Factor VIII lt 10
• Factor IX 124
• Anti-VIII autobodies measurement 20.0 Bethesda
  Units/mL

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Acquired hemophilia A Treatment

• Prednison 1 mg/kg per day
• Cyclophosphamide 0.7 g/m² body surface D1 - D21 -
  D42
• rFVIIa (Novoseven ) 6mg each 3 hours

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Acquired hemophilia A Epidemiology

• Incidence 0.2-1.0 per million per year
• Median age 60 70 years
• Mortality 10 20

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Acquired hemophilia A Etiology
Idiopathic (50) Postpartum (10) Diabetes Autoimm
une disorders SLE Rhumatoid arthritis Multiple
sclerosis Temporal arteritis Sjögren
syndrome Myasthenia gravis Hypothyroïdism Inflam
matory bowel disease Psoriasis, Pemphigus Asthma,
COPD
Acute hepatitis B C Drugs Penicillins Sulfamid
es Phenytoin Methyldopa Interferon
alpha Fludarabin BCG-vaccination Malignancy Sol
id tumors Haematological malignancies ...
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Acquired hemophilia A Treatment
Treatment of bleeding lt 5 BU / mL Human f VIII
concentrates Desmopressin gt 5 BU /
mL Porcine f VIII concentrates Activated
prothrombin complex concentrates Recombinant
actvated factor VII Temporary reduction of
inhibitor titer Plasmapheresis Immunoadsorbtion
of immunoglobulins by polyclonal
immunoglobulins Eradication of the
autoantibody Immunosuppressive agents
corticosteroids, cyclophosphamide,
azat. Second-line therapy ciclosporin, high dose
immunoglobulins Other treatment rituximab,
interferon alpha