Inhibitor eradication in refractory acquired hemophilia with lenalidomide
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Acquired hemophilia A (AHA) is a very rare disease caused by antibodies against factor VIII (FVIII). Standard treatment consists of steroids, cyclophosphamide, and rituximab. However, only 42–70% of all patients achieve long-term remission after first-line treatment [1, 2]. The optimal treatment of refractory AHA is still controversial.
We report on a 66-year-old male patient diagnosed with AHA after spontaneous spinal bleeding resulting in paraplegia. Initial FVIII activity was < 1% and the inhibitor titer 700 Bethesda units (BU). Before the diagnosis of AHA, he was healthy and not taking any medication. Diagnostic workup showed 0.75% atypical B cells in the bone marrow but no cytologic abnormalities and a regular male karyotype. Immunofixation was weakly positive for IgG kappa but serum electrophoresis and free light chains were normal. CT scan did not reveal any sign of an underlying disease and there was no evidence for a rheumatologic disorder.
Compliance with ethical standards
Conflict of interest
The authors declared that they have no conflict of interest.
This article does not contain any studies with human participants performed by any of the authors.
Informed consent was obtained from the patient for being included in the study.
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