Skip to main content

Multicentric Castleman Disease

  • Chapter
  • First Online:
Atlas of Lymph Node Pathology

Abstract

Multicentric Castleman disease (MCD) is a rare, systemic lymphoproliferative disorder associated with human herpesvirus 8 (HHV8) infection. Patients present with lymphadenopathy and systemic symptoms including fever, night sweats, and weight loss. The disease commonly involves peripheral and abdominal lymph nodes as well as the spleen and liver. Laboratory studies often demonstrate elevation in erythrocyte sedimentation rate, C-reactive protein, serum lactate dehydrogenase, and IL-6. Cytopenias, especially anemia and thrombocytopenia, may be present also. Most patients with MCD patients have human immunodeficiency virus (HIV) infection, but a subset of patients have POEMS syndrome (peripheral neuropathy, organomegaly, endocrinopathy, monoclonal M protein, skin lesions) and rare patients have neither HIV infection nor POEMS syndrome.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 299.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Jaye DL, Geigerman CM, Herling M, Eastburn K, Waller EK, Jones D. Expression of the plasmacytoid dendritic cell marker BDCA-2 supports a spectrum of maturation among CD4+ CD56+ hematodermic neoplasms. Mod Pathol. 2006;19:1555–62.

    Article  PubMed  CAS  Google Scholar 

  2. Khoury JD, Medeiros LJ, Manning JT, Sulak LE, Bueso-Ramos C, Jones D. CD56(+) TdT(+) blastic natural killer cell tumor of the skin: a primitive systemic malignancy related to myelomonocytic leukemia. Cancer. 2002;94:2401–8.

    Article  PubMed  CAS  Google Scholar 

  3. Hui PK, Chan JK, Ng CS, Kung IT, Gwi E. Lymphadenopathy of Kimura’s disease. Am J Surg Pathol. 1989;13:177–86.

    Article  PubMed  CAS  Google Scholar 

  4. Kapoor NS, O’Neill JP, Katabi N, Wong RJ, Shah JP. Kimura disease: diagnostic challenges and clinical management. Am J Otolaryngol. 2012;33:259–62.

    Article  PubMed  Google Scholar 

  5. Deshpande AH, Nayak S, Munshi MM, Bobhate SK. Kimura’s disease. Diagnosis by aspiration cytology. Acta Cytol. 2002;46:357–63.

    Article  PubMed  Google Scholar 

  6. Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol. 1969;81:1–14.

    Article  PubMed  CAS  Google Scholar 

  7. Suthaus J, Stuhlmann-Laeisz C, Tompkins VS, Rosean TR, Klapper W, Tosato G, et al. HHV-8-encoded viral IL-6 collaborates with mouse IL-6 in the development of multicentric Castleman disease in mice. Blood. 2012;119:5173–81.

    Article  PubMed  CAS  Google Scholar 

  8. Amin HM, Medeiros LJ, Manning JT, Jones D. Dissolution of the lymphoid follicle is a feature of the HHV8+ variant of plasma cell Castleman’s disease. Am J Surg Pathol. 2003;27:91–100.

    Article  PubMed  Google Scholar 

  9. Dispenzieri A. How I, treat POEMS syndrome. Blood. 2012;119:5650–8.

    Article  PubMed  CAS  Google Scholar 

  10. Kimura T, Yoshhimura S, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissue. Trans Soc Pathol Jpn. 1948;37:179–80.

    Google Scholar 

  11. Day TA, Abreo F, Hoajsoe DK, Aarstad RF, Stucker FJ. Treatment of Kimura’s disease: a therapeutic enigma. Otolaryngol Head Neck Surg. 1995;112:333–7.

    Article  PubMed  CAS  Google Scholar 

  12. Lucioni M, Novara F, Fiandrino G, Riboni R, Fanoni D, Arra M, et al. Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion. Blood. 2011;118:4591–4.

    Article  PubMed  CAS  Google Scholar 

  13. Dupin N, Diss TL, Kellam P, Tulliez M, Du MQ, Sicard D, et al. HHV-8 is associated with a plasmablastic variant of Castleman disease that is linked to HHV-8-positive plasmablastic lymphoma. Blood. 2000;95:1406–12.

    PubMed  CAS  Google Scholar 

  14. Xu X, Fu J, Fang Y, Liang L. Kimura disease in children: a case report and a summary of the literature in Chinese. J Pediatr Hematol Oncol. 2011;33:306–11.

    Article  PubMed  Google Scholar 

  15. Al-Maghrabi J, Kamel-Reid S, Bailey D. Immunoglobulin and T-cell receptor gene rearrangement in Castleman’s disease: molecular genetic analysis. Histopathology. 2006;48:233–8.

    Article  PubMed  CAS  Google Scholar 

  16. Soulier J, Grollet L, Oksenhendler E, Miclea JM, Cacoub P, Baruchel A, et al. Molecular analysis of clonality in Castleman’s disease. Blood. 1995;86:1131–8.

    PubMed  CAS  Google Scholar 

  17. Menke DM, DeWald GW. Lack of cytogenetic abnormalities in Castleman’s disease. South Med J. 2001;94:472–4.

    PubMed  CAS  Google Scholar 

  18. Nakamura H, Nakaseko C, Ishii A, Kogure K, Kawano E, Hashimoto S, et al. Chromosomal abnormalities in Castleman’s disease with high levels of serum interleukin-6 [in Japanese]. [Rinsho ketsueki]. Jap J Clin Hematol. 1993;34:212–7.

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media New York

About this chapter

Cite this chapter

Miranda, R.N., Khoury, J.D., Medeiros, L.J. (2013). Multicentric Castleman Disease. In: Atlas of Lymph Node Pathology. Atlas of Anatomic Pathology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-7959-8_27

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-7959-8_27

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-7958-1

  • Online ISBN: 978-1-4614-7959-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics