Scopus
YÖKSİS Eşleşti
Acquired Hemophilia A In Adults: A Multicenter Study from Turkey
Indian Journal of Hematology and Blood Transfusion · Ocak 2023
YÖKSİS Kayıtları
Acquired Hemophilia A In Adults: A Multicenter Study from Turkey
Indian Journal of Hematology and Blood Transfusion · 2022 SCI-Expanded
DOKTOR ÖĞRETİM ÜYESİ CEM SELİM →
Acquired Hemophilia A In Adults: A Multicenter Study from Turkey
Springer Science and Business Media LLC · 2023 SCI-Expanded
PROFESÖR RAFİYE ÇİFTÇİLER →
Makale Bilgileri
DergiIndian Journal of Hematology and Blood Transfusion
Yayın TarihiOcak 2023
Cilt / Sayfa39 · 107-115
Scopus ID2-s2.0-85146619996
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.
Yazarlar (17)
1
Eren Arslan Davulcu
2
Zühal Demirci
ORCID: 0000-0001-7509-8020
3
Umut Yılmaz
ORCID: 0000-0002-8672-0679
4
Muhlis Cem Ar
ORCID: 0000-0002-0332-9253
5
Hava Üsküdar Teke
ORCID: 0000-0002-4434-4580
6
Volkan Karakuş
ORCID: 0000-0001-9178-2850
7
Rafiye Çiftçiler
ORCID: 0000-0001-5687-8531
8
Cem Selim
ORCID: 0000-0002-2717-8306
9
İrfan Yavaşoğlu
ORCID: 0000-0003-1703-2175
10
Salih Sertaç Durusoy
ORCID: 0000-0002-3577-6330
11
Vahap Okan
ORCID: 0000-0002-9869-8181
12
Aydan Akdeniz
ORCID: 0000-0002-5160-4803
13
Alkım Yolcu
ORCID: 0000-0003-2438-7097
14
Ismet Aydoǧdu
15
Tekin Güney
ORCID: 0000-0001-6313-0361
16
Asu Fergün Yılmaz
ORCID: 0000-0001-5118-6894
17
Fahri Şahin
ORCID: 0000-0001-9315-8891
Anahtar Kelimeler
Acquired coagulation disorders
Hemophilia and other bleeding disorders
Other coagulation inhibitors
Kurumlar
Aksaray Üniversitesi
Aksaray Turkey
Aydin Adnan Menderes University
Aydin Turkey
Bak?rkoy Dr. Sadi Konuk Egitim ve Arast?rma Hastanesi
Istanbul Turkey
Ege University Medical School
Izmir Turkey
Eskişehir Osmangazi Üniversitesi
Eskisehir Turkey
Gaziantep Üniversitesi
Gaziantep Turkey
İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine
Istanbul Turkey
Manisa Celâl Bayar Üniversitesi
Manisa Turkey
Marmara Üniversitesi
Istanbul Turkey
Mersin Üniversitesi
Mersin Turkey
Sanko Üniversitesi
Gaziantep Turkey
University of Health Sciences
Istanbul Turkey
Metrikler
3
Atıf
17
Yazar
3
Anahtar Kelime