The use of cyclophosphamide plus dexamethasone and rituximab (CyDRi) for the treatment of acquired hemophilia A resulted in durable, high complete remission (CR) rates with a milder toxicity profile compared with the more commonly used regimen of prolonged steroids, according to the results of a retrospective study published in the journal Blood.

“CyDRi proved to be an attractive option for the immunosuppression of elderly patients with acquired hemophilia A,” the authors wrote in their report. There is currently no consensus on the optimal first-line treatment, but many clinicians use steroids. The aim of this study was to determine if a combination immunosuppressive regimen could improve efficacy and mitigate the toxicity associated with prolonged steroid exposure.

The single-center, retrospective study evaluated data of 32 patients diagnosed with acquired hemophilia A between 2009 and 2020 who were treated with CyDRi. Patients received at least 1 cycle of treatment, but continued treatment until CR was achieved.

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The median age of the cohort was 77 (range, 53-87) and 56% were female. The mean comorbidity index was 1.6 and the Eastern Cooperative Oncology Group performance status ranged from 1 to 4, with the majority of patients a 1 or 2. There were 59% of patients with no underlying illness, 25% with an autoimmune disorder, and 22% with a neoplastic disease. The initial factor VIII required was a median of 1 IU/dL and the median initial inhibitor was 17 BU. There were 37.5% of patients who presented with active bleeding symptoms.

There was rapid improvement of bleeding, with a median time to bleeding control of 15.5 days (range, 0-429 days).

CR was achieved by 96.8% of patients, with a median time to first CR of 77 days (range, 19-939 days). The median duration of CR was 630 days, with shorter CR duration significantly associated with high comorbidity index (>2; P =.002). The median number of CyDRi cycles required was 1 (range, 1-2), with 69% of patients needing only 1 cycle. At last follow-up, the overall survival was 90.6%, all of whom were in CR.

There were 2 patients who experienced relapse after achieving CR and both were successfully retreated with the CyDRi regimen.

“Side effects were acceptable and seemed fewer compared with commonly used prolonged steroid therapies,” the authors noted. The only adverse events associated with cyclophosphamide was transient, mild cytopenia.

The authors concluded that “the upfront combined regimen, coined CyDRi, was found to be rapidly effective in an elderly acquired hemophilia A population with remarkably low toxicity and good overall survival.”


Simon B, Ceglédi A, Dolgos J, et al. Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen. Blood. 2022;140:1983-1992. doi: 10.1182/blood.2022016873