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. 2025 Nov 29;42(4):315-319.
doi: 10.4274/tjh.galenos.2025.2025.0201. Epub 2025 Aug 27.

Real-World Clinical Outcomes and Prognostic Factors in Acquired Hemophilia A: A Single-Center Retrospective Analysis

Selin Küçükyurt Kaya  1 , Pelin Öztürk  2 , Umut Yılmaz  1 , Duygu Seyhan Erdoğan  2 , Tuğrul Elverdi  1 , Zafer Başlar  1 , Muhlis Cem Ar  1
Affiliations

Real-World Clinical Outcomes and Prognostic Factors in Acquired Hemophilia A: A Single-Center Retrospective Analysis

Selin Küçükyurt Kaya et al. Turk J Haematol. .

Abstract
in English, Turkish

Acquired hemophilia A (AHA) is a rare but potentially life-threatening bleeding disorder. This single-center retrospective study aimed to assess clinical features, treatment strategies, and prognostic indicators in adult AHA patients. Eleven patients diagnosed between 2008 and 2024 were reviewed. Clinical data, laboratory findings, treatments, and outcomes were analyzed. Survival estimates and prognostic factors were evaluated using Kaplan-Meier and univariate analyses. Median age was 41 years; 54.5% of the patients were female. Pregnancy-associated AHA (36.4%) had excellent outcomes with steroid monotherapy and no relapse. Idiopathic and autoimmune cases required combination therapy and had higher relapse rates. The median follow-up duration was 27 months. All patients achieved remission (median response time: 62 days), though 36.4% relapsed. High inhibitor titer (>20 Bethesda unit) predicted delayed response (p=0.038); male sex and major bleeding were linked to shorter relapse-free survival. Baseline inhibitor burden and disease etiology influence AHA prognosis. Tailored therapy and multicenter validation are warranted to refine management strategies.

Edinsel hemofili A (EHA) nadir görülen ancak yaşamı tehdit edebilen bir kanama bozukluğudur. Bu tek merkezli retrospektif çalışmamızın amacı, erişkin EHA hastalarında klinik özellikleri, tedavi stratejilerini ve prognostik göstergeleri değerlendirmektir. 2008-2024 yılları arasında tanı konulan 11 hasta incelendi. Klinik ve laboratuvar verileri, tedaviler ve klinik sonuçlar analiz edildi. Beklenen sağkalımlar ve prognostik faktörler Kaplan-Meier yöntemi ve tek değişkenli analiz ile değerlendirildi. Ortanca yaş 41 idi; %54,5’i kadındı. Gebelikle ilişkili EHA (%36,4) steroid monoterapisine çok iyi yanıt verdi ve nüks gözlenmedi. İdiyopatik ve otoimmün hastalık ilişkili olgularda kombine immünosüpresif tedavi gerekti ve nüks oranı daha yüksekti. Ortanca takip süresi 27 aydı. Tüm hastalarda remisyona ulaşıldı (ortanca yanıt süresi: 62 gün), ancak %36,4’ünde nüks görüldü. Yüksek inhibitör titresi (>20 Bethesda ünitesi) gecikmiş yanıtla (p=0,038); erkek cinsiyet ve majör kanama daha kısa nükssüz sağkalımla ilişkilendirildi. Tanıdaki inhibitör yükü ve hastalık etiyolojisi EHA prognozunu etkilemektedir. Yönetim stratejilerini iyileştirmek için kişiselleştirilmiş tedavi ve çok merkezli validasyon gereklidir.

Keywords: Acquired hemophilia A; Bleeding disorder; Inhibitor; Bypassing agents; Immunosuppressive therapy.

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Conflict of interest statement

No conflict of interest was declared by the authors.

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