Skip to main content

Advertisement

SpringerLink
Log in

Successful Immunomodulatory Therapy in Castleman Disease with Paraneoplastic Pemphigus Vulgaris

  • Original Paper
  • Published:
Pathology & Oncology Research

Abstract

Castleman disease is a rare lymphoproliferative disorder. The clinical signs and symptoms of the disease are primarily mediated by cytokines, especially interleukin-6. We presented the case of a young female. In May 2004, a 30-year-old otherwise healthy looking woman presented with oral ulcerations resistant to topical and systemic antibiotic and antimycotic treatment. Bullous mucosal lichen or pemphigus vulgaris were suspected. Histological examination and direct and indirect immunofluorescence confirmed the diagnosis of pemphigus. Search for neoplasm revealed a retroperitoneal Castleman tumour sized 15 × 6 × 5 cm in the abdominal MRI. The tumour was a bleeder, so the removal was partial. Histological examination showed hyalin hypervascular Castleman disease. Considering her young, fertile age and the multicentric Castleman disease, non-cytostatic immunomodulatory therapy was started including steroid, cyclosporine-A and thalidomide treatment. The control abdominal CT showed a small residual tumour on the bladder. The residual tumour was removed in repeated surgery. At this time the histological examination showed transient type tumour between plasma cell and vascular variant. Currently, i.e. 4 years after the onset of the disease. 18FDG PET/CT examination showed low metabolic active mass in the right iliacal region, but our patient had no symptoms or complaints. She is on 200 mg thalidomide a day and no tumour progression can be seen. Castleman disease can be successfully treated with non-cytostatic immunomodulatory therapy.

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

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8

Similar content being viewed by others

References

  1. Castleman B, Thowne VW (1954) Case records of the Massachuetts General Hospital weekly clinicopathological exercises: Case 40011. N Eng J Med 250:26–30

    Article  Google Scholar 

  2. van den Berge M, Pauwels P, Jakimowicz JJ et al (2002) Hyaline vascular Castleman’s disease: a case report and brief review of the literature. Neth J Med 60(11):444–447, Dec

    PubMed  Google Scholar 

  3. Casper C (2005) The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol 129:3–17

    Article  PubMed  Google Scholar 

  4. Oksenhendler E, Carcelain G, Aoki Y et al (2000) High levels of human herpesvirus 8 viral load, human interleukin-6, interleukin-10, and C reactive protein correlate with exacerbation of multicentric Castleman disease in HIV-infected patients. Blood 96:2069–2073

    CAS  PubMed  Google Scholar 

  5. Waterston A, Bower M (2004) Fifty years of multicentric Castleman’s disease. Acta Oncologica 43:698–704

    Article  PubMed  Google Scholar 

  6. Harris NL, Swerdlow S, Campo E et al (2008) The World Health Organization (WHO) classification of lymphoid neoplasms: what’s new? Ann Oncol 19(4):119

    Google Scholar 

  7. Anhalt GJ, Kim SC, Stanley JR et al (1990) Paraneoplastic pemphigus. An autoimmune mucocutaneus disease associated with neoplasia. N Engl J Med 323:1729–1735

    CAS  PubMed  Google Scholar 

  8. Anhalt GJ (2004) Paraneoplastic pemphigus. J Investig Dermatol Symp Proc 9(1):29–33

    Article  PubMed  Google Scholar 

  9. Dham A, Peterson BA (2007) Castleman disease. Curr Opin Hematol 14:354–359

    Article  CAS  PubMed  Google Scholar 

  10. Chorzelski T, Hashimoto T, Maciejewska B et al (1999) Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. J Am Acad Dermatol 41(3):3939–3400

    Google Scholar 

  11. Nishimoto N, Kanakura Y, Aozasa K et al (2005) Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood 106:2627–2632

    Article  CAS  PubMed  Google Scholar 

  12. Starkey C, Joste N, Lee FC (2006) Near-total resolution of multicentric Castleman disease by prolonged treatment with thalidomid. Am J Haematol 81:303–304 (letter)

    Article  Google Scholar 

  13. Lee FC, Merchant SH (2003) Alleviation of systemic manifestations of multicentric Castleman’s disease by thalidomide. Am J Hematol 73:48–53

    Article  PubMed  Google Scholar 

  14. Nikolskaia OV, Nousari CH, Anhalt GJ (2003) Paraneoplastic pemphigus in association with Castleman’s disease. Br J Dermatol 149:1143–1151

    Article  CAS  PubMed  Google Scholar 

  15. Cunha PR, de Oliveira JR, Salles MJ et al (2004) Pemphigus vulgaris with involvement of the cervix treated using thalidomide therapy. Int J Dermatol 43(9):682–684

    Article  PubMed  Google Scholar 

  16. Hess G, Wagner V, Kreft A et al (2006) Effects of bortezomib on pro-inflammatory cytokine levels and transfusion dependency in a patient with multicentric Castleman disease. Brit J Haematol 134:544–549

    Article  CAS  Google Scholar 

  17. Diéval C, Bonnet F, Mauclére S et al (2007) Multicentric Castleman disease: Use of HHV8 viral load monitoring and positron emission tomography during follow-up. Leuk Lymphoma 48(9):1881–1883

    Article  PubMed  Google Scholar 

  18. Nakamura Y, Tokuyama O, Muso A et al (2002) Asymptomatic pelvic Castleman disease in an infertile woman: case report. Arch Gynecol Obstet 269:156–158

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. 3rd Department of Internal Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary

    Zsofia Miltenyi & Arpad Illes

  2. Department of Oncology, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary

    Judit Toth & Andrea Gonda

  3. Department of Parodontology, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary

    Ildiko Tar

  4. Department of Dermatology, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary

    Eva Remenyik

Authors
  1. Zsofia Miltenyi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Judit Toth
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Andrea Gonda
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ildiko Tar
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Eva Remenyik
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Arpad Illes
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Zsofia Miltenyi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Miltenyi, Z., Toth, J., Gonda, A. et al. Successful Immunomodulatory Therapy in Castleman Disease with Paraneoplastic Pemphigus Vulgaris. Pathol. Oncol. Res. 15, 375–381 (2009). https://doi.org/10.1007/s12253-008-9133-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12253-008-9133-x

Keywords

Search

Navigation