Abstract
An 80-year-old man was treated at our hospital for chronic kidney disease, diabetes mellitus and hypertension. He presented a bleeding tendency over the past several weeks. When he was admitted to our hospital with a consciousness disturbance, he had irregular 10–20 cm patches of subcutaneous bleeding. Laboratory findings showed prolonged activated partial thromboplastin time (APTT) but normal platelet count and prothrombin time. The administration of fresh frozen plasma did not control the bleeding tendency and the result of APTT cross-mixing test suggested the presence of factor VIII inhibitor. The diagnosis of acquired hemophilia A was made by a marked decrease in factor VIII activity levels (<1 %) and a high titer of factor VIII inhibitor (11 Bethesda units). To remove the factor VIII inhibitor and suppress its production, plasma exchange was performed and methylprednisolone was administered. Thereafter, the bleeding tendency could be controlled with APTT measurement normalization. Plasma exchange does not have a first priority in the treatment but our case findings suggested that plasma exchange is an effective method for eliminating factor VIII inhibitor and alleviating acquired hemophilia A severity.
References
Nihon Kessenshiketsu Gakkai Koutensei Ketsuyubyo A shinryo Gaidorain Sakusei Iinkai. Kotensei Ketsuyubyo A shinryo Gaidorain. http://www.jsth.org. Accessed 21 Oct 2014.
Zeitler H, Ulrich-Merzenich G, Hess L, Konsek E, Unkrig C, Walger P, Vetter H, Brackmann HH. Treatment of acquired hemophilia by the Bonn-Malmo Protocol: documentation of an in vivo immunomodulating concept. Blood. 2005;105:2287–93.
Zeitler H, Ulrich-Merzenich G, Marquardt N, Oldenburg J, Goldmann G. immunoabsorption for pregnancy associated severe acquired haemophilia A. Ther Apher Dial. 2014;18:103–10.
Ogata H, Sakai S, Koiwa F, Tayama H, Kinugasa E, Ideura T, Akizawa T. Plasma exchange for acquired hemophilia A. Ther Apher. 1999;3:320–2.
Hay CR, Brown S, Collins PW, Keeling DM, Liesner R. The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation. Br J Haematol. 2006;133:591–605.
Working Group on Acquired Haemophilia of the Nordic Haemophilia Centres. Acquires haemophilia: Nordic Guidelines for diagnosis and treatment. http://www.Medfak.lu.se/pdf/medkirortpdf/koagulation/nordic_guidelines. Accessed 21 Oct 2014.
Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA. Hay CR; UK Haemophilia Centre Doctor’s Organisation. Acquired haemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors Organisation. Blood. 2007;109:1870–7.
Huth-Kühne A, Baudo F, Collins P, Ingerslev J, Kessler CM, Lévesque H, Castellano ME, Shima M, St-Louis J. International recommendation on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica. 2009;94:566–75.
Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A. Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol. 2003;121:21–35.
Collins PW, Percy CL. Advances in the understanding of acquired haemophilia A: implications for clinical practice. Br J Haematol. 2010;148:183–94.
Nagano T, Nakazono K, Iwamoto H, Sato T, Shizume Y, Takenaka Y, Munekata K, Koga N. Comparative study of polyethylene plasma separator sterilized by ethylene oxide and gamma rays. Jpn J Apheresis. 1997;16:297–8.
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Goto, S., Ookawara, S., Ueda, Y. et al. Effectiveness of plasma exchange for acquired type A hemophilia. CEN Case Rep 4, 196–199 (2015). https://doi.org/10.1007/s13730-015-0168-z
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DOI: https://doi.org/10.1007/s13730-015-0168-z