دورية أكاديمية
Acquired hemophilia a in adults: a multicenter study from Turkey
| العنوان: | Acquired hemophilia a in adults: a multicenter study from Turkey |
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| المؤلفون: | Arslan Davulcu, Eren, Demirci, Zühal, Yılmaz, Umut, Ar, Muhlis Cem, Teke, Hava Üsküdar, Karakuş, Volkan, Çiftçiler, Rafiye, Selim, Cem, Yavaşoğlu, İrfan, Durusoy, Salih Sertaç, Okan, Vahap, Akdeniz, Aydan, Yolcu, Alkım, Aydoğdu, İsmet, Güney, Tekin, Yılmaz, Asu Fergün, Şahin, Fahri |
| المساهمون: | Tıp Fakültesi |
| بيانات النشر: | Springer |
| سنة النشر: | 2023 |
| المجموعة: | Aksaray University Institutional Repository (DSpace@Aksaray) |
| مصطلحات موضوعية: | Acquired Coagulation Disorders, Hemophilia and Other Bleeding Disorders, Other Coagulation Inhibitors |
| الوصف: | Acquired hemophilia A (AHA) is a rare disease caused by autoantibodies inhibiting factor VIII (FVIII) activity. Although the conditionis usually idiopathic, there may be other underlying diseases. Treatment consists of two steps: treatment of acute bleeding and immunosuppression. In this multicenter study, we aimed to demonstrate the clinical characteristics, management details, and survival of AHA patients in Turkey. Data was collected from eleven centers in Turkey. aPTT, FVIII, FVIII inhibitor, and hemoglobin (HB) levels, mixing test results, and demographics at diagnosis, treatment information, adverse events, bleeding episodes during follow-up, relapses, and outcome were analyzed. Twenty-nine patients were analyzed (58.6% female). No underlying disorder could be detected in 14 patients. The most prevalent etiologies were pregnancy, malignancy and infections. The median FVIII activity and FVIII inhibitor titer at diagnosis were 0.7% (0.0–29.4%) and 32.6 BU (0.6–135.6 BU) respectively. Bleeding was severe in 44.8% of patients. The HB value was significantly lower in patients with severe bleeding. Most of the patients (n = 25, 86.2%) had only one bleeding episode without relapse, three patients (10.3%) had two bleeding episodes, and one patient had more than three bleedings. 21 (75%) patients received hemostatic therapy. The use of recombinant FVIIa was slightly higher than activated prothrombin complex concentrate (15 versus 10 patients). Immunosuppressive treatment was initiated in 26 (93%) patients. Regimens containing steroid, cyclophosphamide, and rituximab in different combinations were the most preferred. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| تدمد: | 09714502 |
| العلاقة: | Indian Journal of Hematology and Blood Transfusion; Makale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı; https:/dx.doi.org/10.1007/s12288-022-01556-8; https://hdl.handle.net/20.500.12451/10289Test; 39; 107; 115 |
| DOI: | 10.1007/s12288-022-01556-8 |
| الإتاحة: | https://doi.org/20.500.12451/10289Test https://doi.org/10.1007/s12288-022-01556-8Test https://hdl.handle.net/20.500.12451/10289Test |
| حقوق: | info:eu-repo/semantics/closedAccess |
| رقم الانضمام: | edsbas.A526BBB |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 09714502 |
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| DOI: | 10.1007/s12288-022-01556-8 |