PB1233 - A single-center study of reduced-dose corticosteroids therapy for patients with acquired hemophilia A.

Tuesday, June 27, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

Yoshiyuki Ogawa, MD, PhD

Senior Assistant Professor
Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan
Maebashi, Gumma, Japan

Co-Author(s)

KY

Kunio Yanagisawa

Assistant Professor
Infection Control and Prevention Center, Gunma University Hospital
Maebashi, Gumma, Japan
Akira Matsumoto, MD (he/him/his)

Physician
Department of Hematology, Gunma University Graduate School of Medicine, Maebashi, Japan
Maebashi, Gumma, Japan
MK

Mikiya Kajita

doctor
Department of Hematology, Gunma University Graduate School of Medicine
Maebashi, Gumma, Japan
YM

Yuri Miyazawa

Assistant Professor
Department of Hematology, Gunma University Graduate School of Medicine
Maebashi, Gumma, Japan
NK

Nobuhiko Kobayashi

doctor
Department of Hematology, Gunma University Graduate School of Medicine
Maebashi, Gumma, Japan
TO

Tsukasa Osaki

Assistant Professor
Yamagata University School of Medicine
Yamagata, Yamagata, Japan
MS

Masayoshi Souri

Associate Professor
Yamagata University School of Medicine
Yamagata, Yamagata, Japan
AI

Akitada Ichinose

Professor
Yamagata University School of Medicine
Yamagata, Yamagata, Japan
HH

Hiroshi Handa

Associate Professor
Department of Hematology, Gunma University Graduate School of Medicine
Maebashi, Gumma, Japan

Background: Acquired hemophilia A (AHA) is a life-threatening bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). The patient should be placed on immunosuppressive therapy (IST) comprising corticosteroids either alone or in combination with effective cytotoxic agents such as cyclophosphamide, cyclosporine or, rituximab. Through these therapeutic interventions, 70-80% of patients with AHA reportedly achieve complete remission (CR).

Aims: However, about a quarter of patients die of infections associated with IST, so there is a need to reduce the toxicity of IST.

Methods: We treated a total of 25 patients with AHA (median age, 78 years; range, 30-92 years; 15 males) between October 2011 and September 2021 at Gunma University Hospital. Coagulative CR (cCR) was defined FVIII activity >60 IU/dl and negative results for FVIII inhibitor. Considering advanced age, cognitive impairment, history of thrombosis, and comorbidities such as hypertension and diabetes, the attending physician decided to reduce the dose of IST.

Results: Twenty-two cases received IST, of which 21 patients were treated with prednisolone alone, except for one patient who received rituximab monotherapy for gastrointestinal ulcer. Only 6 patients (28.6%; median age, 72.5 years; median FVIII inhibitor 35.0 BU/ml) received full doses (1 mg/kg) of steroid therapy. The remaining 15 patients (71.4%) were received reduced-dose steroid therapy, 9 of whom (median age, 76 years; median FVIII inhibitor 29.0 BU/ml) received dose reductions of 0.6-0.9 mg/kg and 6 patients (median age, 83 years; median FVIII inhibitor 7.45 BU/ml) received 0.5 mg/kg of prednisolone. All 21 patients achieved cCR in median of 39 days (range, 8-163 days). The median time to cCR was 44 days, 39 days, and 49 days, respectively, and there was no statistically significant difference between the three groups (p = 0.957).

Conclusion(s): Reduced-dose corticosteroids therapy for AHA patients can be expected to be as effective as standard treatment, under the appropriate case selection.