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SCI-Expanded Özgün Makale Scopus
Acquired Hemophilia A In Adults: A Multicenter Study from Turkey
Springer Science and Business Media LLC 2023
Scopus Eşleşmesi Bulundu
3
Atıf
39
Cilt
107-115
Sayfa
Scopus Yazarları: Eren Arslan Davulcu, Zühal Demirci, Umut Yılmaz, Muhlis Cem Ar, Hava Üsküdar Teke, Volkan Karakuş, Cem Selim, Ismet Aydoǧdu, Tekin Güney, Asu Fergün Yılmaz, Fahri Şahin, Rafiye Çiftçiler, İrfan Yavaşoğlu, Salih Sertaç Durusoy, Vahap Okan, Aydan Akdeniz, Alkım Yolcu
Özet
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. The median follow-up period was 13 months (2–156 months). Median overall survival was 154.97 months. Four and six-year survival were 90.9 ± 0.8% and 77.9 ± 14.1% respectively. This is a unique study that investigated the demographic characteristics, treatment approaches, and patient survival of AHA in Turkey.
Anahtar Kelimeler (Scopus)
Acquired coagulation disorders Hemophilia and other bleeding disorders Other coagulation inhibitors

Anahtar Kelimeler

Acquired coagulation disorders Hemophilia and other bleeding disorders Other coagulation inhibitors

Makale Bilgileri

Dergi Springer Science and Business Media LLC
ISSN 0971-4502
Yıl 2023 / 1. ay
Makale Türü Özgün Makale
Hakemlik Hakemli
Endeks SCI-Expanded
Yayın Dili İngilizce
Kapsam Uluslararası
Toplam Yazar 17 kişi
Erişim Türü Elektronik
Erişim Linki Makaleye Git
Alan Temel Alan

YÖKSİS Yazar Kaydı

Yazar Adı Davulcu Eren Arslan, Demirci Zuhan, YILMAZ UMUT, AR MUHLİS CEM, ÜSKÜDAR TEKE HAVA, KARAKUŞ VOLKAN, ÇİFTÇİLER RAFİYE, SELİM CEM, YAVAŞOĞLU İRFAN, DURUSOY SALİH SERTAÇ, OKAN VAHAP, AKDENİZ AYDAN, YOLCU ALKIM, AYDOĞDU İSMET, GÜNEY TEKİN, YILMAZ ASU FERGÜN, ŞAHİN FAHRİ
YÖKSİS ID 6791633