Volume 24, Issue 10 , Pages 548-552, October 2008
Cytologic Diagnosis of Primary Effusion Lymphoma in an HIV-Negative Patient
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
- Human herpesvirus 8 (HHV-8)-associated peritoneal primary effusion lymphoma (PEL) in two HIV-negative elderly patients . Am J Hematol . 2004;76:88–91
- Human herpes virus-8 associated primary effusion lymphoma of the pleural cavity in HIV-negative elderly men . Eur Respir J . 1999;14:1231–1234
- Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus . Blood . 1996;88:645–656
- Kaposi's sarcoma-associated herpesvirus DNA sequences in AIDS-related and AIDS-unrelated lymphomatous effusions . Br J Haematol . 1996;94:533–543
- 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
- Human herpesvirus 8-associated primary effusion lymphoma in HIV-negative patients: a clinicoepidemiologic variant resembling classic Kaposi's sarcoma . Haematologica . 2002;87:339–343
- 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
- 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
- . 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
- Primary effusion lymphoma (PEL) in HIV-negative patients—a distinct clinical entity . Leuk Lymphoma . 2001;41:439–443
- Combined chemotherapy including high-dose methotrexate in KSHV/HHV8-associated primary effusion lymphoma . Am J Hematol . 2003;73:143–148
- Human-immunodeficiency-virus-negative, human-herpes-virus-8-negative abdominal cavity primary effusion lymphoma . Clin Oncol (R Coll Radiol) . 2005;17:636–638
- 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
- Detection of HHV-8/KSHV DNA sequences in AIDS-associated extranodal lymphoid malignancies . Leukemia . 1996;10:1358–1362
- . Serous effusions in malignant lymphomas: a review . Diagn Cytopathol . 2006;34:335–347
- 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
- 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
- Association of Kaposi's sarcoma-associated herpesvirus-positive primary effusion lymphoma with expression of the CD138/syndecan-1 antigen . Blood . 1997;90:4894–4900
- . Primary effusion lymphoma: a liquid phase lymphoma of fluid-filled body cavities . Adv Cancer Res . 2001;80:115–146
- Human herpes virus-8 associated with two cases of primary-effusion lymphoma . Ann Hematol . 2000;79:336–339
- HHV8-negative primary effusion lymphoma of the peritoneal cavity presenting with a distinct immunohistochemical phenotype . Pathol Int . 2001;51:293–300
- 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
- Kaposi's sarcomaassociated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas . N Engl J Med . 1995;332:1186–1191
- Primary body cavity-based AIDS-related lymphomas . Am J Clin Pathol . 1996;105:221–229
- The natural history and molecular heterogeneity of HIV-associated primary malignant lymphomatous effusions . J Acquir Immune Defic Syndr Hum Retrovirol . 1996;13:215–226
- 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
- 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
© 2008 Elsevier. Published by Elsevier Inc. All rights reserved.
Volume 24, Issue 10 , Pages 548-552, October 2008
