Kaohsiung Journal of Medical Sciences
Volume 24, Issue 10 , Pages 548-552, October 2008

Cytologic Diagnosis of Primary Effusion Lymphoma in an HIV-Negative Patient

  • Yue-Chiu Su

      Affiliations

    • Department of Pathology, Kaohsiung Medical University Hospital, Taipei, Taiwan
  • ,
  • Chee-Yin Chai

      Affiliations

    • Department of Pathology, Kaohsiung Medical University Hospital, Taipei, Taiwan
    • Department of Pathology, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taipei, Taiwan
  • ,
  • Shih-Sung Chuang

      Affiliations

    • Department of Pathology, Chi-Mei Medical Center, Tainan, Taipei, Taiwan
    • Department of Pathology, Taipei Medical University, Taipei, Taiwan
  • ,
  • Yung-Liang Liao

      Affiliations

    • Department of Pathology, Chi-Mei Medical Center, Tainan, Taipei, Taiwan
  • ,
  • Wan-Yi Kang

      Affiliations

    • Department of Pathology, Kaohsiung Medical University Hospital, Taipei, Taiwan
    • Department of Pathology, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taipei, Taiwan
    • Corresponding Author InformationAddress correspondence and reprint requests to: Dr Wan-Yi Kang, Department of Pathology, Kaohsiung Medical University Hospital, 100 Tzyou 1st Road, Kaohsiung 807, Taiwan

Received 3 March 2008; accepted 26 March 2008.

Article Outline

Primary effusion lymphoma (PEL) is an unusual and rare type of non-Hodgkin's lymphoma characterized by lymphomatous effusion of pleural, pericardial or peritoneal cavities without lymphadenopathy or organomegaly. It is associated with human herpes virus-8 (HHV-8) and occurs most often in immunodeficient patients. We present a case of PEL in a 69-year-old male presenting with pleural effusion and ascites. Fluid aspiration showed a monomorphic population of atypical lymphoid cells, which were medium- to large-sized, with mono- or binucleated hyper-chromatic nuclei and a small to moderate amount of basophilic cytoplasm containing cytoplasmic vesicles. Immunohistochemically, the lymphoid cells expressed CD138 and multiple myeloma oncogene 1, were positive for HHV-8, and were monoclonal for immunoglobulin heavy chain gene rearrangement. They were negative for Epstein-Barr virus by in situ hybridization. Unfortunately, the patient died during the first course of chemotherapy with cyclophosphamide, vincristine and prednisone.

Key Words:  human herpes virus-8 , human immunodeficiency virus , primary effusion lymphoma

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References 

  1. Boulanger E , Hermine O , Fermand JP , et al.   Human herpesvirus 8 (HHV-8)-associated peritoneal primary effusion lymphoma (PEL) in two HIV-negative elderly patients . Am J Hematol . 2004;76:88–91
  2. Ascoli V , Scalzo CC , Danese C , et al.   Human herpes virus-8 associated primary effusion lymphoma of the pleural cavity in HIV-negative elderly men . Eur Respir J . 1999;14:1231–1234
  3. Nador RG , Cesarman E , Chadburn A , et al.   Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus . Blood . 1996;88:645–656
  4. Carbone A , Gloghini A , Vaccher E , et al.   Kaposi's sarcoma-associated herpesvirus DNA sequences in AIDS-related and AIDS-unrelated lymphomatous effusions . Br J Haematol . 1996;94:533–543
  5. 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
  6. Ascoli V , Lo Coco F , Torelli G , et al.   Human herpesvirus 8-associated primary effusion lymphoma in HIV-negative patients: a clinicoepidemiologic variant resembling classic Kaposi's sarcoma . Haematologica . 2002;87:339–343
  7. 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
  8. Cobo F , Hernandez S , Hernandez L , et al.   Expression of potentially oncogenic HHV-8 genes in an EBV-negative primary effusion lymphoma occurring in an HIV-seronegative patient . J Pathol . 1999;189:288–293
  9. Banks PM , Warnke RA . Primary effusion lymphoma . In:  Jaffe ES ,  Harris NL ,  Stein H , et al. editor. Tumours of Haematopoietic and Lymphoid Tissues. World Health Organization Classification of Tumours . Lyon: IARC Press; 2001;p. 179–180
  10. Klepfish A , Sarid R , Shtalrid M , et al.   Primary effusion lymphoma (PEL) in HIV-negative patients—a distinct clinical entity . Leuk Lymphoma . 2001;41:439–443
  11. Boulanger E , Daniel MT , Agbalika F , et al.   Combined chemotherapy including high-dose methotrexate in KSHV/HHV8-associated primary effusion lymphoma . Am J Hematol . 2003;73:143–148
  12. Jenkins C , Sorour Y , Blake E , et al.   Human-immunodeficiency-virus-negative, human-herpes-virus-8-negative abdominal cavity primary effusion lymphoma . Clin Oncol (R Coll Radiol) . 2005;17:636–638
  13. DePond W , Said JW , Tasaka T , et al.   Kaposi's sarcomaassociated herpesvirus and human herpesvirus 8 (KSHV/HHV8)-associated lymphoma of the bowel. Report of two cases in HIV-positive men with secondary effusion lymphomas . Am J Surg Pathol . 1997;21:719–724
  14. Otsuki T , Kumar S , Ensoli B , et al.   Detection of HHV-8/KSHV DNA sequences in AIDS-associated extranodal lymphoid malignancies . Leukemia . 1996;10:1358–1362
  15. Das DK . Serous effusions in malignant lymphomas: a review . Diagn Cytopathol . 2006;34:335–347
  16. Brimo F , Michel RP , Khetani K , et al.   Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis . Cancer . 2007;111:224–233
  17. Carbone A , Gloghini A , Cozzi MR , et al.   Expression of MUM1/IRF4 selectively clusters with primary effusion lymphoma among lymphomatous effusions: implications for disease histogenesis and pathogenesis . Br J Haematol . 2000;111:247–257
  18. Gaidano G , Gloghini A , Gattei V , et al.   Association of Kaposi's sarcoma-associated herpesvirus-positive primary effusion lymphoma with expression of the CD138/syndecan-1 antigen . Blood . 1997;90:4894–4900
  19. Gaidano G , Carbone A . Primary effusion lymphoma: a liquid phase lymphoma of fluid-filled body cavities . Adv Cancer Res . 2001;80:115–146
  20. Niitsu N , Chizuka A , Sasaki K , et al.   Human herpes virus-8 associated with two cases of primary-effusion lymphoma . Ann Hematol . 2000;79:336–339
  21. Tanaka S , Katano H , Tsukamoto K , et al.   HHV8-negative primary effusion lymphoma of the peritoneal cavity presenting with a distinct immunohistochemical phenotype . Pathol Int . 2001;51:293–300
  22. Gaidano G , Capello D , Cilia AM , et al.   Genetic characterization of HHV-8/KSHV-positive primary effusion lymphoma reveals frequent mutations of BCL6: implications for disease pathogenesis and histogenesis . Genes Chromosomes Cancer . 1999;24:16–23
  23. Cesarman E , Chang Y , Moore PS , et al.   Kaposi's sarcomaassociated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas . N Engl J Med . 1995;332:1186–1191
  24. Ansari MQ , Dawson DB , Nador R , et al.   Primary body cavity-based AIDS-related lymphomas . Am J Clin Pathol . 1996;105:221–229
  25. Komanduri KV , Luce JA , McGrath MS , et al.   The natural history and molecular heterogeneity of HIV-associated primary malignant lymphomatous effusions . J Acquir Immune Defic Syndr Hum Retrovirol . 1996;13:215–226
  26. Halfdanarson TR , Markovic SN , Kalokhe U , et al.   A non-chemotherapy treatment of a primary effusion lymphoma: durable remission after intracavitary cidofovir in HIV negative PEL refractory to chemotherapy . Ann Oncol . 2006;17:1849–1850
  27. Luppi M , Trovato R , Barozzi P , et al.   Treatment of herpesvirus associated primary effusion lymphoma with intracavity cidofovir . Leukemia . 2005;19:473–476

PII: S1607-551X(09)70015-4

doi:10.1016/S1607-551X(09)70015-4

Kaohsiung Journal of Medical Sciences
Volume 24, Issue 10 , Pages 548-552, October 2008