Skip to main content

Advertisement

Log in

Disappearance of malignant cells by effusion drainage alone in two patients with HHV-8-unrelated HIV-negative primary effusion lymphoma-like lymphoma

  • Case Report
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin lymphoma usually confined to the body cavities of predominantly immunosuppressed patients infected with human herpes virus-8 (HHV-8). In PEL, malignant cells are usually negative for B-cell markers, such as CD19, CD20, and CD79a, but are positive for activation and plasma cell-related markers, such as CD30, CD38, and CD138. It has been reported that HHV-8-unrelated PEL shows high expression of B-cell markers, which is referred to as PEL-like lymphoma. Here, we report two cases of HHV-8-unrelated HIV-negative PEL-like lymphoma in which malignant cells regressed spontaneously after effusion drainage alone. These cells expressed B-cell markers, such as CD20. No chemotherapy was given to either patient, as they were of an advanced age, and both achieved a complete response by effusion drainage alone. One showed a complete response for 16 months after effusion drainage, and the other has survived for 11 months with a complete response. This suggests that sufficient drainage of serous effusion may induce and maintain a complete response in some patients with HHV-8-unrelated HIV-negative PEL-like lymphoma, which represents an excellent treatment option for elderly patients with this disease.

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

Access this article

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

Similar content being viewed by others

References

  1. Said J, Ceaarman E. Primary effusion lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2008. p. 260–1.

    Google Scholar 

  2. Kobayashi Y, Kamitsuji Y, Kuroda J, Tsunoda S, Uoshima N, Kimura S, et al. Comparison of human herpes virus 8 related primary effusion lymphoma with human herpes virus 8 unrelated primary effusion lymphoma-like lymphoma on the basis of HIV: report of 2 cases and review of 212 cases in the literature. Acuta Haematol. 2007;117:132–44.

    Article  Google Scholar 

  3. Adiguzel C, Bozkurt SU, Kaygusuz I, Uzay A, Tecimer T, Bayik M. Human herpes virus 8-unrelated primary effusion lymphoma-like lymphoma: report of a rare case and review of the literature. APMIS. 2009;117:222–9.

    Article  PubMed  Google Scholar 

  4. Carbone A, Globhini A. PEL and HHV-unrelated effusion lymphomas: classification and diagnosis. Cancer. 2008;114:225–7.

    Article  PubMed  Google Scholar 

  5. Ichinohasama R, Miura I, Kobayashi N, Saitoh Y, DeCoteau JF, Saiki Y, et al. Herpes virus type 8-negative primary effusion lymphoma associated with PAX-5 gene rearrangement and hepatitis C virus: a case report and review of the literature. Am J Surg Pathol. 1998;22:1528–37.

    Article  CAS  PubMed  Google Scholar 

  6. Saiki M, Saitoh T, Inoue M, Hatta Y, Yamazaki T, Itoh T, et al. Human herpesvirus-8 negative primary effusion lymphoma with complete clinical remission after removal of ascites. Rinsho Ketsueki. 2002;43:548–53. Japanese.

    PubMed  Google Scholar 

  7. Inoue S, Miyamoto T, Yoshino T, Yamadori I, Hagari Y, Yamamoto O. Primary effusion lymphoma with skin involvement. J Clin Pathol. 2006;59:1221–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Terasaki Y, Okumura H, Saito K, Sato Y, Yoshino T, Ichinohasama R, Ishida Y. HHV-8/KSHV-negative and CD20-positive primary effusion lymphoma successfully treated by pleural drainage followed by chemotherapy containing rituximab. Intern Med. 2008;47:2175–8.

    Article  PubMed  Google Scholar 

  9. Suzuki K, Ino K, Sugawara Y, Mizutani M, Sekine T, Katayama N. Prolonged survival in a patient with human herpesvirus-8-negative primary effusion lymphoma after combination chemotherapy with rituximab. Gan To Kagaku Ryoho. 2008;35:691–4. Japanese.

    PubMed  Google Scholar 

  10. De Filippi R, Iaccarino G, Frigeri F, Di Francia R, Crisci S, Capobianco G, et al. Elevation of clonal serum free light chains in patients with HIV-negative primary effusion lymphoma (PEL) and PEL-like lymphoma. Br J Haematol. 2009;147:405–8.

    Article  PubMed  Google Scholar 

  11. Tsagarakis NJ, Argyrou A, Gortzolidis G, Kentrou N, Papadhimitriou SI, Tzanetou K, et al. Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia. Int J Hematol. 2009;90:94–8.

    Article  PubMed  Google Scholar 

  12. Taira T, Nagasaki A, Okudaira T, Miyagi T, Iha K, Takushi Y, et al. HIV- and HHV-8-negative primary effusion lymphoma-like lymphoma presenting with lymphomatous effusions complicated by cardiac tamponade-a case report. Gan To Kagaku Ryoho. 2009;36:1195–8. Japanese.

    PubMed  Google Scholar 

  13. Takahashi T, Hangaishi A, Yamamoto G, Ichikawa M, Imai Y, Kurokawa M. HIV-negative, HHV-8-unrelated primary effusion lymphoma-like lymphoma: report of two cases. Am J Hematol. 2010;85:85–7.

    PubMed  Google Scholar 

  14. Kagoya Y, Takahashi T, Yoshimoto T, Ichikawa M, Hangaishi A, Fukayama M, et al. Recurrent pericardial effusion after treatment for primary effusion lymphoma-like lymphoma: an autopsied case. Ann Hematol. 2011;90:219–20.

    Article  PubMed  Google Scholar 

  15. Sandra Horning J, Saul Rosenberg A. The natural history of initially untreated low-grade non-Hodgkin’s lymphomas. N Engl J Med. 1984;311:1471–5.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yasushi Terasaki.

About this article

Cite this article

Terasaki, Y., Yamamoto, H., Kiyokawa, H. et al. Disappearance of malignant cells by effusion drainage alone in two patients with HHV-8-unrelated HIV-negative primary effusion lymphoma-like lymphoma. Int J Hematol 94, 279–284 (2011). https://doi.org/10.1007/s12185-011-0906-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-011-0906-8

Keywords

Navigation