Healthcare - West Virginia State Medical Association

| Scientific ArticleA repeat lupus anticoagulant at6 weeks remained negative.Case 2: Acquired Hemophiliawith CompartmentSyndromeThe patient is a 61 year oldwoman who presented initiallywith hematomas of the left andright forearm following intravenousblood draws. She developedcompartment syndrome of the rightforearm and underwent surgicalrelease at an outside facility withexcessive bleeding. She was foundto have prolonged activated partialthromboplastin time. On transfer toour center, she was noted to havea Factor VIII of 6% and Bethesdatiter of 5-10 units. No underlyingcause for her inhibitor was found.She received treatment with NovoSeven (Recombinant Factor VIIa),intravenous immunoglobulin, andoral prednisone for her Factor VIIIinhibitor. She did well on taperingdoses of prednisone until the dosewas lowered to 2.5 mg a day with theFactor VIII level decreasing to 31%.The prednisone dosage wasincreased to 5 mg. bid withnormalization of the Factor VIII levelto 90%. The prednisone was increasedto 10 mg in the morning and 5 mgin the evening prior to right forearmrevision surgery seven months afterher initial presentation. She safelyunderwent a right wrist Guyon canalrelease, right carpal tunnel releaseand extensive neurolysis to the rightforearm for a distance of 10 cm atboth the medial and ulnar nerve. Scarrevision of the hypertrophic keloidtype scar in the volar right forearm10-11 cm was also performed withconsiderable benefit to the patient.Cyclophosphamide 100 mg.daily was added to prednisone.Her PTT and Factor VIII levelsremained normal. Subsequently,prednisone was tapered off whilecyclophosphamide was continuedfor a total of 10 months. A mildneutropenia corrected after stoppingthe cyclophosphamide. FactorVIII and PTT have remainednormal with no recurrence ofher inhibitor with follow up 3years after initial presentation.DiscussionAcquired inhibitors of coagulationare most often due to antibodiesthat either inhibit the activity orincrease the clearance of a clottingfactor. The most common autoantibodies affecting clotting factoractivity leading to bleeding aredirected against the activity of FactorVIII, a condition called acquiredhemophilia A. 1-3 Patients withacquired hemophilia A can produceantibodies of different IgG subclassesdirected against different epitopeson the Factor VIII molecule. 4-6Collins et al 7-8 have reported anincidence of 1.3-1.5 cases per millionThe sensible choicefor specialized care.Providing comprehensive pediatric and adult ear, noseand throat care, Eye & Ear Clinic Physicians also offerscomplete hearing aid services and allergy testing.304.343.EECP(3327) | eecpwv.comFeaturing an extensive selection oF digitalhearing aid devices and accessories FromNovember/December 2010 | Vol. 106 33