Abstract
Primary effusion lymphoma (PEL) is a recently recognized disease that occurs most often in immunosuppressed patients, either with human immunodeficiency virus (HIV) or in the posttransplantation setting, and it occasionally occurs in nonim-munosuppressed patients. Patients present with lymphomatous effusions in serous cavities—pleura, pericardium, or peritoneum–without any identifiable tumor mass. PEL rarely responds to systemic chemotherapy, and the prognosis is poor, with a median survival time of less than 6 months for most cohorts. A standard treatment for PEL has not yet been identified. We describe a patient with HIV-seronegative PEL who relapsed after combination chemotherapy and then underwent successful treatment with high-dose chemotherapy (HDC) and autologous stem cell transplantation (ASCT).The treatment was well tolerated, and the patient has been in remission for 12 months after HDC and ASCT.
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Cesarman E, Chang Y, Moore PS, Said JW, Knowles DM. Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. N Engl J Med. 1995;332:1186–1191.
Nador RG, Cesarman E, Chadmanburn A, et al. Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi’s sarcoma-associated herpes virus. Blood. 1996;88:645–656.
Fujiwara T, Ichinohasama R, Miura I, et al. Primary effusion lym-phoma of the pericardial cavity carrying t(1;22)(q21;q11) and t(14;17)(q32;q23). Cancer Genet Cytogenet. 2005;156:49–53.
Cobo F, Hernandez S, Hernandez L, et al. Expression of potentially oncogenic HHV-8 genes in an EBV-negative primary effusion lym-phoma occurring in an HIV-seronegative patient. J Pathol. 1999;189:288–293.
Teruya-Feldstein J, Zauber P, Setsuda JE, et al. Expression of human herpesvirus-8 oncogene and cytokine homologues in an HIV-seronegative patient with multicentric Castleman’s disease and primary effusion lymphoma. Lab Invest. 1998;78:1637–1642.
Ohshima K, Ishiguro M, Yamasaki S, et al. Chromosomal and comparative analyses of HHV-8-negative primary effusion lymphoma in five HIV-negative Japanese patients. Leuk Lymphoma. 2002;43:595–601.
Boulanger E,Agbalika F, Maarek O, et al. A clinical, molecular and cytogenetic study of 12 cases of human herpesvirus 8 associated primary effusion lymphoma in HIV-infected patients. Hematol J. 2001;2:172–179.
Klepfish A, Sarid R, Shtalrid M, Shvidel L, Berrebi A, Schattner A. Primary effusion lymphoma (PEL) in HIV-negative patients: a distinct clinical entity. Leuk Lymphoma. 2001;41:439–443.
Waddington TW, Aboulafia DM. Failure to eradicate AIDS-associated primary effusion lymphoma with high-dose chemotherapy and autologous stem cell reinfusion: case report and literature review. AIDS Patient Care STDS. 2004;18:67–73.
Knowles DM, Inghirami G, Ubriaco A, Dalla-Favera R. Molecular genetic analysis of three AIDS-associated neoplasms of uncertain lineage demonstrates their B-cell derivation and the possible patho-genetic role of the Epstein-Barr virus. Blood. 1989;73:792–799.
Gessain A, Briere J, Angelin-Duclos C, et al. Human herpes virus 8 (Kaposi’s sarcoma herpes virus) and malignant lymphoproliferations in France: a molecular study of 250 cases including two AIDS-associated body cavity based lymphomas. Leukemia. 1997;11:266–272.
Ansari MQ, Dawson DB, Nador R, et al. Primary body cavity-based AIDS-related lymphomas. Am J Clin Pathol. 1996;105:221–229.
Cesarman E, Moore PS, Rao PH, Inghirami G, Knowles DM, Chang Y. In vitro establishment and characterization of two acquired immunodeficiency syndrome-related lymphoma cell lines (BC-1 and BC-2) containing Kaposi’s sarcoma-associated her-pesvirus-like (KSHV) DNA sequences. Blood. 1995;86:2708–2714.
Banks PM, Warnke RA. Primary effusion lymphoma. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, eds. World Health Organization Classification of Tumours. Pathology & Genetics: Tumours of Haematopoietic and Lymphoid Tissues.Lyon, France: IARC Press;2001:179–180.
Sarid R, Klepfish A, Schattner A. Virology, pathogenetic mechanisms, and associated diseases of Kaposi sarcoma-associated herpesvirus (human herpesvirus 8). Mayo Clin Proc. 2002;77:941–949.
Said JW, Tasaka T, Takeuchi S, et al. Primary effusion lymphoma in women: report of two cases of Kaposi’s sarcoma herpes virus-associated effusion-based lymphoma in human immunodeficiency virus-negative women. Blood. 1996;88:3124–3128.
Strauchen JA, Hauser AD, Burstein D, Jimenez R, Moore PS, Chang Y. Body cavity-based malignant lymphoma containing Kaposi sarcoma-associated herpesvirus in an HIV-negative man with previous Kaposi sarcoma. Ann Intern Med. 1996;125:822–825.
Boulanger E, Daniel MT, Agbalika F, Oksenhendler E. Combined chemotherapy including high-dose methotrexate in KSHV/HHV8-associated primary effusion lymphoma. Am J Hematol. 2003;73:143–148.
Krishnan A, Molina A, Zaia J, et al. Autologous stem cell transplantation for HIV-associated lymphoma. Blood. 2001;98:3857–3859.
Re A, Cattaneo C, Michieli M, et al. High-dose therapy and autologous peripheral-blood stem-cell transplantation as salvage treatment for HIV-associated lymphoma in patients receiving highly active antiretroviral therapy. J Clin Oncol. 2003;21:4423–4427.
Kang EM, de Witte M, Malech H, et al. Nonmyeloablative conditioning followed by transplantation of genetically modified HLA-matched peripheral blood progenitor cells for hematologic malignancies in patients with acquired immunodeficiency syndrome. Blood. 2002;99:698–701.
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Won, JH., Han, SH., Bae, SB. et al. Successful Eradication of Relapsed Primary Effusion Lymphoma with High-Dose Chemotherapy and Autologous Stem Cell Transplantation in a Patient Seronegative for Human Immunodeficiency Virus. Int J Hematol 83, 328–330 (2006). https://doi.org/10.1532/IJH97.A30510
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DOI: https://doi.org/10.1532/IJH97.A30510