Acquired hemophilia a in adults: a multicenter study from Turkey

Basit Öğe Kaydı
dc.contributor.authorArslan Davulcu, Eren
dc.contributor.authorDemirci, Zühal
dc.contributor.authorYılmaz, Umut
dc.contributor.authorAr, Muhlis Cem
dc.contributor.authorTeke, Hava Üsküdar
dc.contributor.authorKarakuş, Volkan
dc.contributor.authorÇiftçiler, Rafiye
dc.contributor.authorSelim, Cem
dc.contributor.authorYavaşoğlu, İrfan
dc.contributor.authorDurusoy, Salih Sertaç
dc.contributor.authorOkan, Vahap
dc.contributor.authorAkdeniz, Aydan
dc.contributor.authorYolcu, Alkım
dc.contributor.authorAydoğdu, İsmet
dc.contributor.authorGüney, Tekin
dc.contributor.authorYılmaz, Asu Fergün
dc.contributor.authorŞahin, Fahri
dc.date.accessioned2023-02-24T07:49:01Z
dc.date.available2023-02-24T07:49:01Z
dc.date.issued2023
dc.departmentTıp Fakültesi
dc.description.abstractAcquired 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.
dc.identifier.doi10.1007/s12288-022-01556-8
dc.identifier.endpage115en_US
dc.identifier.issn00971-4502
dc.identifier.issue1en_US
dc.identifier.scopusqualityQ3
dc.identifier.startpage107en_US
dc.identifier.urihttps:/dx.doi.org/10.1007/s12288-022-01556-8
dc.identifier.urihttps://hdl.handle.net/20.500.12451/10289
dc.identifier.volume39en_US
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherSpringer
dc.relation.ispartofIndian Journal of Hematology and Blood Transfusion
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subjectAcquired Coagulation Disorders
dc.subjectHemophilia and Other Bleeding Disorders
dc.subjectOther Coagulation Inhibitors
dc.titleAcquired hemophilia a in adults: a multicenter study from Turkey
dc.typeArticle

Dosyalar

Orijinal paket
Listeleniyor 1 - 1 / 1
[ X ]
İsim:
arslan davulcu-eren-2023.pdf
Boyut:
376.52 KB
Biçim:
Adobe Portable Document Format
Açıklama:
Tam Metin / Full Text
İndir
Lisans paketi
Listeleniyor 1 - 1 / 1
[ X ]
İsim:
license.txt
Boyut:
1.44 KB
Biçim:
Item-specific license agreed upon to submission
Açıklama:
İndir

Koleksiyon

Makale Koleksiyonu
PubMed İndeksli Yayınlar Koleksiyonu
Scopus İndeksli Yayınlar Koleksiyonu