![]() ![]() Holstein K, Liu X, Smith A, Knobl P, Klamroth R, Geisen U, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Knoebl P, Marco P, Baudo F, Collins P, Huth-Kuhne A, Nemes L, et al. Acquired hemophiliaAin the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’Organisation. 2012 38:447-53.Ĭollins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, et al. Acquired Inhibitors of Coagulation Factors: Part II. Autoantibodies to coagulation factors: From pathophysiology to diagnosis and therapy. ![]() 2015 40(3).Ĭugno M, Gualtierotti R, Tedeschi A, Meroni PL. Clinical and Laboratory Approaches to Hemophilia A. Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. Moreover, therapeutical choice and side effects control require special consideration to achieve hemostasis and durable remission.Ĭollins P, Baudo F, Huth-Kühne A, Ingerslev J, Kessler CM, Castellano MEM, et al. Treatment for acquired hemophilia A encompasses hemostatic therapy, immunosuppression, and treatment of underlying disease. ![]() However, several challenges lay within patients’ management strategy, such as diagnosis workup and therapeutical choices. Prolonged activated partial thromboplastin time, uncorrected mixing test, low FVIII activity, and detection of FVIII inhibitors support the diagnosis of acquired Hemophilia A. Hemophilia A occurs due to the development of an antibody against FVIII, moreover up to half of these cases have underlying conditions. Pemilihan terapi serta pengendalian terhadap efek samping dari pengobatan memerlukan perhatian khusus agar tercapai hemostasis dan remisi yang bertahan lama.Īcquired Hemophilia A can potentially cause life-threatening conditions due to profuse bleeding, but this autoimmune disease is mostly underdiagnosed. Rintangan dalam manajemen pasien dimulai dari penegakan diagnosis hingga penentuan terapi, baik terapi hemostatik, imunosupresi, serta pengobatan penyakit penyerta. Pemanjangan activated partial thromboplastin time, mixing test yang tidak terkoreksi, rendahnya aktivitas FVIII, dan bukti inhibitor FVIII mendukung penegakan diagnosis Hemofilia A yang didapat. Penyakit autoimun akibat pembentukan autoantibodi (inhibitor) terhadap FVIII ini hampir setengahnya memiliki gangguan lain yang mendasari. Hemofilia A yang didapat adalah penyakit yang jarang terdiagnosis dan seringkali salah terdiagnosis namun berpotensi menyebabkan perdarahan yang mengancam nyawa. ![]()
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