Skip to main content
SpringerLink
Log in

A case of essential mixed cryoglobulinemia and associated acquired von-Willebrand disease treated with rituximab

  • Published:
Journal of Thrombosis and Thrombolysis Aims and scope Submit manuscript

    We’re sorry, something doesn't seem to be working properly.

    Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

Abstract

Current treatment options of essential mixed cryoglobulinemia (EMC); include immunosuppressive approaches, such as corticosteroids, cyclophosphamide, plasma exchange, other cytotoxic drugs in moderate to severe manifestations. Some controlled studies have been carried out to assess the efficacy of anti-CD20 monoclonal antibody, rituximab in patients with hepatitis C (HCV) related cryoglobulinemia (CG) and in patients with autoimmune disorders. Recent trials and some case reports demonstrate a beneficial role for rituximab in HCV related mixed CG. Although, the published evidence for treatment of EMC with rituximab is restricted to case reports, which have shown positive results. Several diseases include lymphoproliferative and myeloproliferative disorders, solid tumors, immunological disorders, cardiovascular disorders and some drugs associated with acquired von Willebrand disease (avWD). CG, which is a kind of immune complex disease, may be related with development of autoantibodies to various autoantigens. In this present case report, we showed the efficacy of rituximab in a 21-year-old female patient, suffered from neuropathy and arthralgia related with EMC, and developed avWD, presented with mucosal bleeding associated with CG. von Willebrand factor activity of our patient also increased with controlling the underlying disease, EMC by rituximab. This case demonstrate that rituximab may be an effective treatment option in EMC and avWD mainly related to CG.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Rego TC, Massumoto CM, Batista RS, Moura LH, Soares LM, Gomes AP (2007) The use of monoclonal antibody (rituximab) in the treatment of type II mixed cryoglobulinemia. Braz J Infect Dis 11(1):174–175

    Article  PubMed  Google Scholar 

  2. Franchini M, Lippi G (2007) Acquired von Willebrand syndrome: an update. Am J Hematol 82(5):368–375

    Article  PubMed  CAS  Google Scholar 

  3. De Vita S, Quartuccio L, Fabris M (2007) Rituximab in mixed cryoglobulinemia: increased experience and perspectives. Dig Liver Dis 39(Suppl 1):S122–S128

    PubMed  Google Scholar 

  4. Arzoo K, Sadeghi S, Liebman HA (2002) Treatment of refractory antibody mediated autoimmune disorders with an anti-CD20 monoclonal antibody (rituximab). Ann Rheum Dis 61:922–924

    Article  PubMed  CAS  Google Scholar 

  5. Nehme-Schuster H, Korganow AS, Pasquali JL, Martin T (2005) Rituximab inefficiency during type 1 cryoglobulinaemia. Rheumatology 44:410–411

    Article  PubMed  CAS  Google Scholar 

  6. Cohen H, Green S, Jones S, Amos N, William BD (2007) Lack of efficacy of rituximab in a patient with essential mixed cryoglobulinaemia. Rheumatology (Oxford) 46(2):366–367

    Article  CAS  Google Scholar 

  7. Visentini M, Granata M, Veneziano ML, Borghese F, Carlesimo M, Pimpinelli F, Fiorilli M, Casato M (2007) Efficacy of low-dose rituximab for mixed cryoglobulinemia. Clin Immunol 125(1):30–33

    Article  PubMed  CAS  Google Scholar 

  8. Eby CS (2007) Bleeding and thrombosis risks in plasma cell dyscrasias. Hematol Am Soc Hematol Educ Prog 2007:158–164

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Hematology, Medical Faculty, Dicle University, 21280, Diyarbakir, Turkey

    Semir Pasa, Abdullah Altintas, Orhan Ayyildiz & Ekrem Muftuoglu

  2. Department of Oncology, Medicine Faculty, Dicle University, Diyarbakir, Turkey

    Timucin Cil

  3. Department of Nephrology, Medicine Faculty, Dicle University, Diyarbakir, Turkey

    Ramazan Danis

Authors
  1. Semir Pasa
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Abdullah Altintas
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Timucin Cil
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ramazan Danis
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Orhan Ayyildiz
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ekrem Muftuoglu
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Semir Pasa.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Pasa, S., Altintas, A., Cil, T. et al. A case of essential mixed cryoglobulinemia and associated acquired von-Willebrand disease treated with rituximab. J Thromb Thrombolysis 27, 220–222 (2009). https://doi.org/10.1007/s11239-008-0195-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11239-008-0195-0

Keywords

Search

Navigation