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Successful rituximab treatment of an elderly Japanese patient with HHV8-positive, HIV-negative multicentric Castleman disease

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Abstract

Human herpesvirus-8 (HHV8)-positive, human immunodeficiency virus (HIV)-negative multicentric Castleman disease (MCD) is a rare and age-related lymphoproliferative disorder caused by cytokine storm. Rituximab treatment is currently recommended because B-cell depletion eliminates the primary reservoir for HHV8. We report the first case of effective rituximab treatment of a Japanese patient (an 87-year-old woman) with this disorder. Her inflammatory symptoms and lymphadenopathy improved after medium-dose steroid therapy, but these symptoms recurred during steroid tapering. After one course of rituximab therapy, she achieved sustained remission. HHV8-associated MCD should be considered as a possible diagnosis in HIV-negative patients with inflammatory symptoms and lymphadenopathy.

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References

  1. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135:1353–64.

    Article  Google Scholar 

  2. Wu YJ, Su KY. Updates on the diagnosis and management of multicentric Castleman disease. Tzu Chi Med J. 2021;33:22–8.

    Article  Google Scholar 

  3. Munshi N, Mehra M, van de Velde H, Desai A, Potluri R, Vermeulen J. Use of a claims database to characterize and estimate the incidence rate for Castleman disease. Leuk Lymphoma. 2015;56:1252–60.

    Article  Google Scholar 

  4. Masaki Y, Kawabata H, Fujimoto S, Kawano M, Iwaki N, Kotani T, et al. Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan. J Clin Exp Hematop. 2019;59:175–8.

    Article  Google Scholar 

  5. Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy. Blood. 2014;123:2924–33.

    Article  CAS  Google Scholar 

  6. Katano H, Sata T. Human herpesvirus 8—virology, epidemiology and related diseases. Jpn J Infect Dis. 2000;53:137–55.

    CAS  PubMed  Google Scholar 

  7. Dossier A, Meignin V, Fieschi C, Boutboul D, Oksenhendler E, Galicier L. Human herpesvirus 8-related Castleman disease in the absence of HIV infection. Clin Infect Dis. 2013;56:833–42.

    Article  CAS  Google Scholar 

  8. Fujii T, Taguchi H, Katano H, Mori S, Nakamura T, Nojiri N, et al. Seroprevalence of human herpesvirus 8 in human immunodeficiency virus 1-positive and human immunodeficiency virus 1-negative populations in Japan. J Med Virol. 1999;57:159–62.

    Article  CAS  Google Scholar 

  9. Suda T, Katano H, Delsol G, Kakiuchi C, Nakamura T, Shiota M, et al. HHV-8 infection status of AIDS-unrelated and AIDS-associated multicentric Castleman’s disease. Pathol Int. 2001;51:671–9.

    Article  CAS  Google Scholar 

  10. Fajgenbaum DC, June CH. Cytokine storm. N Engl J Med. 2020;383:2255–73.

    Article  CAS  Google Scholar 

  11. Marietta M, Pozzi S, Luppi M, Bertesi M, Cappi C, Morselli M, et al. Acquired haemophilia in HIV negative HHV-8 positive multicentric Castleman’s disease: a case report. Eur J Haematol. 2003;70:181–2.

    Article  Google Scholar 

  12. Pastor MA, Vasco B, Mosquera JM, Debén G, Bautista P, Requena L. Two HHV8-related illnesses in a HIV-negative patient: Kaposi’s sarcoma and multicentric Castleman’s disease. Response to treatment with rituximab and CHOP. Actas Dermosifiliogr. 2006;97:385–90 (in Spanish with English abstract).

    Article  CAS  Google Scholar 

  13. Cervera Grau JM, Galiana Esquerdo G, Llorca Ferrándiz C, Briceño García H, Díaz Castellano M, Férriz MP. Complete remission in a pancytopenic HIV negative, HHV-8 positive patient with multicentric Castleman’s disease induced with anti-CD20. Clin Transl Oncol. 2006;8:540–1.

    Article  CAS  Google Scholar 

  14. Fragasso A, Mannarella C, Ciancio A, Calvario A, Scarasciulli ML. Complete remission and virologic response to combined chemoimmunotherapy (R-CVP) followed by rituximab maintenance in HIV-negative, HHV-8 positive patient with multicentric Castleman disease. Leuk Lymphoma. 2008;49:2224–6 (letter).

    Article  Google Scholar 

  15. Nicoli P, Familiari U, Bosa M, Allice T, Mete F, Morotti A, et al. HHV8-positive, HIV-negative multicentric Castleman’s disease: early and sustained complete remission with rituximab therapy without reactivation of Kaposi sarcoma. Int J Hematol. 2009;90:392–6.

    Article  CAS  Google Scholar 

  16. Law AB, Ryan G, Lade S, Prince HM. Development of Kaposi’s sarcoma after complete remission of multicentric Castlemans disease with rituximab therapy in a HHV8-positive HIV-negative patient. Int J Hematol. 2010;91:347–8 (letter).

    Article  Google Scholar 

  17. Műzes G, Sipos F, Csomor J, Sréter L. Successful tocilizumab treatment in a patient with human herpesvirus 8-positive and human immunodeficiency virus-negative multicentric Castleman’s disease of plasma cell type nonresponsive to rituximab-CVP therapy. APMIS. 2013;121:668–74.

    Article  Google Scholar 

  18. Kantarci FE, Eren R, Gündoğan C, Huq GE, Doğu MH, Suyanı E. A HHV-8 positive, HIV negative multicentric Castleman disease treated with R-CEOP chemotherapy and valganciclovir combination. J Infect Chemother. 2016;22:483–5.

    Article  Google Scholar 

  19. Murphy C, Hawkes E, Chionh F, Chong G. Durable remission of both multicentric Castleman’s disease and Kaposi’s sarcoma with valganciclovir, rituximab and liposomal doxorubicin in an HHV-8-positive, HIV-negative patient. J Clin Pharm Ther. 2017;42:111–4.

    Article  CAS  Google Scholar 

  20. Kounatidis D, Rontogianni D, Sampaziotis D, Vardaka M, Giatra C, Dolapsakis C, et al. An HHV-8 positive HIV negative multicentric Castleman’s disease, who responded well to rituximab alone. Cardiovasc Hematol Disord Drug Targets. 2020;20:84–6.

    Article  CAS  Google Scholar 

  21. 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.

    Article  CAS  Google Scholar 

  22. Cattani P, Cerimele F, Porta D, Graffeo R, Ranno S, Marchetti S, et al. Age-specific seroprevalence of human herpesvirus 8 in Mediterranean regions. Clin Microbiol Infect. 2003;9:274–9.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Hematology, Nephrology and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita City, Akita, 010-8543, Japan

    Hajime Kaga, Honami Kurahashi & Naoto Takahashi

  2. Department of Hematology, Nephrology and Rheumatology, Noshiro Kosei Medical Center, Noshiro, Japan

    Arisa Kubota & Yoshiaki Hatano

  3. Division of Clinical Pathology, Akita University Hospital, Akita, Japan

    Hiroshi Nanjo

  4. Department of Life Science, Akita University Graduate School of Engineering Science, Akita, Japan

    Hideki Wakui

Authors
  1. Hajime Kaga
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  2. Honami Kurahashi
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  3. Arisa Kubota
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  4. Yoshiaki Hatano
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  5. Hiroshi Nanjo
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  6. Hideki Wakui
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  7. Naoto Takahashi
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Correspondence to Hajime Kaga.

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Conflict of interest

Dr. Naoto Takahashi reports that he received grants and personal fees from Novartis Pharmaceuticals, personal fees from Bristol Myers Squibb, grants and personal fees from Pfizer, grants and personal fees from Otsuka Pharmaceutical, and grants from Kyowa Kirin, Astellas Pharma, Chugai Pharmaceutical, Asahi Kasei Pharma, Ono Pharmaceutical, and Eisai Pharmaceuticals outside of the submitted work. The remaining authors state that they have no COI.

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Kaga, H., Kurahashi, H., Kubota, A. et al. Successful rituximab treatment of an elderly Japanese patient with HHV8-positive, HIV-negative multicentric Castleman disease. Int J Hematol 115, 129–134 (2022). https://doi.org/10.1007/s12185-021-03222-7

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  • DOI: https://doi.org/10.1007/s12185-021-03222-7

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