Title: The most expensive sore throat I have ever seen'''
1The most expensive sore throat I have ever seen...
2Clinical 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)
3Clinical 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
4Clinical 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
5Clinical 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
6Clinical 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
7Acquired 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
8Acquired hemophilia A Epidemiology
- Incidence 0.2-1.0 per million per year
- Median age 60 70 years
- Mortality 10 20
9Acquired 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 ...
10Acquired 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