Acquired Hemophilia A In Adults: A Multicenter Study from Turkey

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dc.authoridARSLAN DAVULCU, EREN/0000-0001-9262-2883
dc.authoriddurusoy, salih sertac/0000-0002-3577-6330
dc.contributor.authorDavulcu, Eren Arslan
dc.contributor.authorDemirci, Zuhal
dc.contributor.authorYilmaz, Umut
dc.contributor.authorAr, Muhlis Cem
dc.contributor.authorTeke, Hava Uskudar
dc.contributor.authorKarakus, Volkan
dc.contributor.authorCiftciler, Rafiye
dc.date.accessioned2023-01-12T19:51:31Z
dc.date.available2023-01-12T19:51:31Z
dc.date.issued2022
dc.departmentN/A/Departmenten_US
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. 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.en_US
dc.identifier.doi10.1007/s12288-022-01556-8
dc.identifier.issn0971-4502
dc.identifier.issn0974-0449
dc.identifier.issn0971-4502en_US
dc.identifier.issn0974-0449en_US
dc.identifier.urihttps://doi.org/10.1007/s12288-022-01556-8
dc.identifier.urihttps://hdl.handle.net/11454/76288
dc.identifier.wosWOS:000834001400001en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.language.isoenen_US
dc.publisherSpringer Indiaen_US
dc.relation.ispartofIndian Journal Of Hematology And Blood Transfusionen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectAcquired coagulation disordersen_US
dc.subjectHemophilia and other bleeding disordersen_US
dc.subjectOther coagulation inhibitorsen_US
dc.subjectFactor-Viiien_US
dc.subjectInhibitorsen_US
dc.titleAcquired Hemophilia A In Adults: A Multicenter Study from Turkeyen_US
dc.typeArticleen_US

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