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Inhibitor eradication in refractory acquired hemophilia with lenalidomide

  • C. PfrepperEmail author
  • W. Poenisch
  • M. Pierer
  • M. Metze
  • T. Kaiser
  • S. Petros
Letter to the Editor
  • 39 Downloads

Dear Editor,

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.

Inhibitor eradication...

Notes

Compliance with ethical standards

Conflict of interest

The authors declared that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants performed by any of the authors.

Informed consent

Informed consent was obtained from the patient for being included in the study.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of HemostaseologyUniversity Hospital LeipzigLeipzigGermany
  2. 2.Division of Hematology and Oncology LeipzigUniversity Hospital LeipzigLeipzigGermany
  3. 3.Division of RheumatologyUniversity Hospital LeipzigLeipzigGermany
  4. 4.Department of CardiologyUniversity Hospital LeipzigLeipzigGermany
  5. 5.Institute of Laboratory MedicineUniversity Hospital LeipzigLeipzigGermany

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