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Acquired Hemophilia A In Adults: A Multicenter Study from Turkey
Indian Journal of Hematology and Blood Transfusion 2023 Cilt 39 Sayı 1
Scopus Eşleşmesi Bulundu
7
Atıf
39
Cilt
107-115
Sayfa
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.
Web of Science Eşleşmesi Bulundu
4
WoS Atıf
39
Cilt
Article
Belge Türü
Kaynak: INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION · s. 107-115
Anahtar Kelimeler (WoS)

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Scimago Dergi Bilgisi Otomatik ISSN Eşleştirmesi 2023 yılı verileri
Indian Journal of Hematology and Blood Transfusion
Q3
SJR Quartile
0,272
SJR Skoru
24
H-Index
Kategoriler: Hematology (Q3)
Alanlar: Medicine
Ülke: India · Springer India
Bu bilgiler makale yılına göre Scimago veritabanından ISSN eşleştirmesiyle otomatik getirilmektedir. Dergi sıralama verileri Scimago'nun ilgili yılı baz alınmaktadır.

Anahtar Kelimeler

WoS | Bir kelimeye tıklayıp ilgili kaynaktaki yayınları görün.

Makale Bilgileri

Dergi Indian Journal of Hematology and Blood Transfusion
ISSN 0971-4502
Yıl 2023 / 1. ay
Cilt / Sayı 39 / 1
Sayfalar 107 – 115
Makale Türü Özgün Makale
Hakemlik Hakemli
Endeks SCI-Expanded
JCR Quartile Q4
Teşvik Puanı 0,27 · YÖKSİS Akademik Teşvik
Yayın Dili İngilizce
Kapsam Uluslararası
Toplam Yazar 17 kişi
Erişim Türü Basılı+Elektronik
Alan Sağlık Bilimleri Temel Alanı Hematoloji (İç Hastalıkları)

YÖKSİS Yazar Kaydı

Yazar Adı ARSLAN DAVULCU EREN,DEMİRCİ ZÜHAL,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 8207330

Metrikler

Scopus Atıf 7
Havuz Atıfları 0
JCR Quartile Q4
Teşvik Puanı 0,27
Yazar Sayısı 17