Acquired hemophilia A and delta storage pool deficiency in a patient with indolent non-Hodgkin lymphoma

Abstract

B-cell lymphoproliferative diseases may be associated with acquired hemostasis disorders, such as acquired hemophilia A (AHA) caused by autoantibodies that neutralize factor VIII activity, and δ-storage pool deficiency, an abnormality of platelet function due to defective dense granules and impaired secretion. We describe the case of a 67-year-old man in whom these two acquired bleeding disorders were concomitantly present as the first clinical manifestation of an indolent non-Hodgkin lymphoma. Immunosuppressive therapy with prednisone was initially started to eradicate anti-FVIII antibodies, subsequently boosted with cyclophosphamide and rituximab, these medications being also chosen to treat the associated indolent lymphoma. Bleeding symptoms were first tackled with limited benefit by using rFVIIa and then rescued using recombinant porcine FVIII. After a 6 month’s follow-up lymphoma and AHA were in remission and platelet function was improved. This case underlines the need of multiple and complex diagnostic and therapeutic approaches to rare acquired bleeding disorders associated with lymphoproliferative diseases.

Keywords:

• Acquired hemophilia A
• bleeding
• hemostatic disorders
• lymphoma
• platelet dysfunction

Acknowledgements

“The authors would like to thank the Italian Ministry of Health - Bando Ricerca Corrente”

Authorship Contribution

RR, RC, and FP designed the study; SMS recruited the patient; RR, CN, AL, SLM,EAF and NA extracted or generated clinical or experimental data; RR, FP and GR interpreted the results; RR, RC and FP wrote the manuscript; all authors read and approved the final version of the manuscript.

Disclosure of Conflicts of Interest

FP reports speaker fees from Bioverativ, Grifols, Roche, Sanofi, Sobi, Spark, and Takeda, outside the submitted work

Supplementary Material

Supplemental data for this article can be accessed on the publisher’s website.