Abstract
Background
Acquired hemophilia A (AHA) is an uncommon coagulation disorder caused by the development of autoantibodies against coagulation factor VIII (FVIII). While intracranial hemorrhage is a known complication of AHA, intracranial hemorrhage as the presenting manifestation of AHA has only been described in three previous case reports.
Method
We report a case of an 86-year-old woman with no previously reported history of coagulopathy presenting with an acute intraparenchymal cerebellar hemorrhage and laboratory studies demonstrating an isolated prolonged activated partial thromboplastin time (aPTT). We discuss an approach to the prolonged aPTT, and review the literature concerning the diagnosis and treatment of AHA.
Results
Occipital decompressive craniectomy with evacuation of the hemorrhage was performed. Eight hours following the procedure, the patient’s status acutely declined with demonstration of a reoccurrence of the cerebellar hemorrhage and new right frontal lobe hemorrhage. After discussion with the patient’s family, life-sustaining support measures were withdrawn. Postmortem analysis revealed a low FVIII activity level and the presence of FVIII inhibitor.
Conclusion
The presentation of intracranial hemorrhage with an isolated prolonged aPTT is concerning for an acquired hemophilia with FVIII deficiency. Other causes of isolated prolonged aPTT such as a lupus anticoagulant must also be considered. Preoperative identification and work-up of the coagulation abnormality is essential to guide initial treatment.
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References
Franchini M, Gandini G, Di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol. 2005;80:55–63.
Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood. 2008;112:250–5.
Bouvry P, Recloux P. Acquired hemophilia. Haematologica. 1994;79:550–6.
Mashiko R, Yamamoto T, Sato M, Noguchi S, Matsumura A. Acquired hemophilia first manifesting as life-threatening intracranial hemorrhage: case report. Neurol Med Chir (Tokyo). 2009;49:93–5.
Bonnaud I, Saudeau D, de Toffol B, Autret A. Recurrence of spontaneous subdural haematoma revealing acquired haemophilia. Eur Neurol. 2003;49:253–4.
Marquardt L, Haubelt H, Gass S, Grau AJ. Intracranial bleeding in acquired hemophilia. Nervenarzt. 2006;77:1480–2.
Kamal AH, Tefferi A, Pruthi RK. How to interpret and pursue an abnormal prothrombin time, activated partial thromboplastin time, and bleeding time in adults. Mayo Clin Proc. 2007;82:864–73.
Chng WJ, Sum C, Kuperan P. Causes of isolated prolonged activated partial thromboplastin time in an acute care general hospital. Singapore Med J. 2005;46:450–6.
Hay CR. Acquired haemophilia. Baillieres Clin Haematol. 1998;11:287–303.
Hartmann M, Sucker C. Pharmacology and clinical use of recombinant activated factor seven in neurosciences. Neurocrit Care. 2007;6:149–57.
Schmidt ML, Gamerman S, Smith HE, Scott JP, DiMichele DM. Recombinant activated factor VII (rFVIIa) therapy for intracranial hemorrhage in hemophilia A patients with inhibitors. Am J Hematol. 1994;47:36–40.
Collins P, Baudo F, Huth-Kuhne A, et al. Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Res Notes. 2010;3:161.
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Micic, D., Williams, E.C. & Medow, J.E. Cerebellar hemorrhage as a first presentation of acquired Hemophilia A. Neurocrit Care 15, 170–174 (2011). https://doi.org/10.1007/s12028-010-9489-0
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DOI: https://doi.org/10.1007/s12028-010-9489-0