Acquired Hemophilia A In Adults: A Multicenter Study from Turkey

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dc.contributor.authorArslan Davulcu E.
dc.contributor.authorDemirci Z.
dc.contributor.authorYılmaz U.
dc.contributor.authorAr M.C.
dc.contributor.authorTeke H.Ü.
dc.contributor.authorKarakuş V.
dc.contributor.authorÇiftçiler R.
dc.contributor.authorSelim C.
dc.contributor.authorYavaşoğlu İ.
dc.contributor.authorDurusoy S.S.
dc.contributor.authorOkan V.
dc.contributor.authorAkdeniz A.
dc.contributor.authorYolcu A.
dc.contributor.authorAydoğdu İ.
dc.contributor.authorGüney T.
dc.contributor.authorYılmaz A.F.
dc.contributor.authorŞahin F.
dc.date.accessioned2024-07-22T08:03:28Z
dc.date.available2024-07-22T08:03:28Z
dc.date.issued2023
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. © 2022, The Author(s), under exclusive licence to Indian Society of Hematology and Blood Transfusion.
dc.identifier.DOI-ID10.1007/s12288-022-01556-8
dc.identifier.issn09714502
dc.identifier.urihttp://akademikarsiv.cbu.edu.tr:4000/handle/123456789/12284
dc.language.isoEnglish
dc.publisherSpringer
dc.subjectactivated prothrombin complex
dc.subjectblood clotting factor 7a
dc.subjectblood clotting factor 8
dc.subjectblood clotting factor 8 inhibitor
dc.subjectcyclophosphamide
dc.subjecthemoglobin
dc.subjectimmunoglobulin
dc.subjectmethylprednisolone
dc.subjectrecombinant blood clotting factor 8
dc.subjectrituximab
dc.subjectactivated partial thromboplastin time
dc.subjectadult
dc.subjectadverse event
dc.subjectaged
dc.subjectaging
dc.subjectArticle
dc.subjectbleeding
dc.subjectclinical article
dc.subjectdemographics
dc.subjectdisease severity
dc.subjectenzyme activity
dc.subjectfemale
dc.subjectfollow up
dc.subjecthealth care management
dc.subjecthemoglobin blood level
dc.subjecthemophilia A
dc.subjecthemostatic technique
dc.subjecthuman
dc.subjectimmunosuppressive treatment
dc.subjectinfection rate
dc.subjectmale
dc.subjectmalignant neoplasm
dc.subjectmiddle aged
dc.subjectoverall survival
dc.subjectpregnancy
dc.subjectprevalence
dc.subjectprotein blood level
dc.subjectrecurrent disease
dc.subjectretrospective study
dc.subjectsex ratio
dc.subjectsurvival factor
dc.subjectTurkey (republic)
dc.subjectvery elderly
dc.subjectyoung adult
dc.titleAcquired Hemophilia A In Adults: A Multicenter Study from Turkey
dc.typeArticle

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