Primary Effusion Lymphoma

Ioakim-Liossi, A.; Mikou, Panagiota; Botsoli-Stergiou, Efi; Petrakakou, E.; Athanassiadou, Pauline; Kordosis, Theodoros; Panagiotakopoulos, George; Karakitsos, Petros

doi:10.1159/000326330

Cited by 10 publications

(11 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Two hundred and twelve cases in 99 papers [1,2,3,4,5,6,7,8,9,10,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,9...…”

Section: Resultsmentioning

confidence: 99%

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

Kobayashi

Kamitsuji²,

Kuroda

et al. 2006

Acta Haematol

View full text Add to dashboard Cite

Background: Primary lymphomatous effusion is a rare lymphoma that arises in the body cavity and has a peculiar proliferative form, lacking a tumor. This primary lymphomatous effusion includes human herpes virus 8 (HHV8)-related primary effusion lymphoma (PEL) and HHV8-unrelated PEL-like lymphoma. We attempted to clarify the nature of the primary lymphomatous effusion. Methods: Using ‘PEL’ and ‘body cavity-based lymphoma’ (BCBL) as key words, reports written in English were collected from PubMed. Primary lymphomatous effusion was defined as BCBL with primary effusion and without tumor at onset. Adding our 2 PEL-like lymphoma cases, each case was studied as to the patients’ and lymphomas’ characteristics, therapy and survival time. Moreover, each item was compared among four groups according to the presence of HHV8 and HIV. Results: In 214 cases investigated, there was no difference in proliferation, but an apparent difference in age, gender, phenotype, effectiveness and prognosis among the four groups. Conclusions: Both PEL and PEL-like lymphoma are thought to be characterized by a peculiar proliferation, regardless of the presence of HHV8. Dividing PEL or PEL-like lymphoma into two subgroups on the basis of HIV presentation might also be appropriate.

show abstract

Section: Resultsmentioning

confidence: 99%

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

Kobayashi

Kamitsuji²,

Kuroda

et al. 2006

Acta Haematol

View full text Add to dashboard Cite

show abstract

“…Cytology is the most common method of diagnosing PEL since nodal and organ involvement are characteristically absent. 13 The majority of patients with PEL are homosexual men who have AIDS or have been HIV seropositive for several years. While most cases of AIDS-related PEL occur in men, it is not exclusively related to homosexuality.…”

Section: Discussionmentioning

confidence: 99%

“…It is not uncommon to see mitotic figures and apoptosis. 9,11,13,19 Reactive cells such as small lymphocytes, histiocytes, and rare mesothelial cells may be admixed with the tumor cells. 23…”

Section: Cytologic Findingsmentioning

confidence: 99%

“…In rare patients, no site other than the affected body cavity appears to be involved. Although a variety of lymphomas has been reported as confined to an effusion, [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] the World Health Organization Classification of Hematolymphoid Neoplasms reserves the term primary effusion lymphoma (PEL) for those associated with human herpesvirus 8 (HHV-8), which usually occur in the setting of immunodeficiency. 20 …”

mentioning

confidence: 99%

See 1 more Smart Citation

Primary Effusion Lymphoma

Caraway

Stewart

2006

Pathology Case Reviews

View full text Add to dashboard Cite

Primary effusion lymphoma (PEL) is a rare, aggressive lymphoma that involves 1 or more of the serous (pleural, peritoneal, and peritoneal) cavities without other tumor masses and is associated with the human herpesvirus 8 (HHV-8), often in the setting of immunodeficiency. PEL occurs predominantly in men with acquired immunodeficiency syndrome (AIDS) but can be seen in iatrogenically immunosuppressed transplant patients and elderly individuals. The effusions show a dispersed population of large pleomorphic cells with eccentrically located nuclei, 1 or more prominent nucleoli, a perinuclear pallor, and moderately abundant cytoplasm. Binucleated and multinucleated cells and mitotic figures are seen commonly. This lymphoma frequently has an indeterminate immunophenotype that is not clearly B cell or T cell in origin, but it is often positive for CD45, CD30, and epithelial membrane antigen. In addition to HHV-8 positivity, many patients also have seropositive results for HIV and Epstein-Barr virus. PEL usually exhibits a complex karyotype without a characteristic aberration. The differential diagnosis typically includes other high-grade lymphomas that occur in HIVinfected patients, immunosuppressed patients, and elderly individuals. The prognosis of this high-grade lymphoma is usually poor, with a survival of only a few months, even with chemotherapy.

show abstract

“…Body cavity based lymphoma is usually associated with primary effusion lymphoma, a distinct clinicopathologic entity linked to human herpes virus 8 and associated with human immunodeficiency virus [3]. While we found several previously reported cases in the literature with the primary presenting symptom of lymphoma as ascites, only two cases were diagnosed as LPL/WM [4 -6].…”

mentioning

confidence: 90%

Lymphoplasmacytic lymphoma presenting as malignant ascites

Ells

Lazarchick

2006

Leukemia & Lymphoma

View full text Add to dashboard Cite

Lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia (LPL/WM) is a distinct clinicopathologic entity consisting of bone marrow infiltration by small lymphocytes, lymphoplasmacytoid lymphocytes and plasma cells with a corresponding IgM paraprotein. The most common presenting symptoms include fatigue, shortness of breath, various symptoms associated with anemia, and an increased bleeding tendency. Small subsets of patients have symptoms of a true hyperviscosity syndrome including mucosal bleeding, retinal hemorrhage and neurologic symptoms. LPL/WM typically occurs in an older population and has an indolent course with a median survival of 5 -10 years [1,2].We report a case of a 57-year-old woman who presented 7 years after orthotopic liver transplant for primary biliary cirrhosis (PBC) with new onset fatigue and massive ascites. Laboratory evaluation revealed aspartate aminotransferase (AST) and alanine aminotransferase (ALT) to be in the normal range (30 IU/L and 19 IU/L, respectively), total bilirubin in the normal range at 0.8 mg/dl, and alkaline phosphatase elevated at 234 IU/L. Serology showed negative hepatitis C virus antibody and positive hepatitis B surface antigen. Her immunosuppression was tacrolimus 3 mg twice a day. Serum protein electrophoresis (SPEP) revealed a monoclonal IgM kappa protein at a level of 2.72 g/dl. The IgM kappa was also present in the ascitic fluid at a level of 1.3 g/dl. The presence of a monoclonal IgM protein was clinically suspicious for a post-transplant lymphoproliferative disorder (PTLD), specifically LPL/WM. Initial ascitic fluid cytology, flow cytometry, and bone marrow biopsy were nondiagnostic for lymphoma.Over the next several months, the patient's ascites did not improve and paraprotein levels remained stable. Repeated ascitic fluid cytology and flow cytometry studies were negative. Serum viscosity was in the normal range at 1.5 cp. Epstein-Barr virus (EBV) DNA quantitation was 3036 copies/ml which was considered elevated but not remarkably so. Tacrolimus was decreased to 1 mg twice a day with the suspicion of PTLD. A liver biopsy showed recurrent PBC with bridging fibrosis, no evidence of lymphoma, and EBV immunohistochemistry was negative.Five months after initial presentation, the patient's serum monoclonal IgM level increased to 3.61 gm/ dl. Repeated EBV titers showed a decrease in copy number to 1412 copies/ml despite an increased paraprotein. Ascitic fluid cytology now showed atypical cells, but remained nondiagnostic ( Figure 1A). Ascitic fluid flow cytometry identified a population of large B cells positive for CD45, CD19, dim CD20, and dim CD38 with kappa light chain restriction. The cells were negative for CD10, CD5, CD23, CD7, CD2, and CD1. Plasma cells with kappa light chain restriction were also present. Repeat bone marrow biopsy now displayed paratrabecular and intertrabecular aggregates of lymphocytes and plasma cells which comprised approximately 50% of the specimen ( Figure 1B, 1C). Immunohistochemical stains on the bone marrow included a CD138 whi...

show abstract

Primary Effusion Lymphoma

Cited by 10 publications

References 10 publications

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

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

Primary Effusion Lymphoma

Lymphoplasmacytic lymphoma presenting as malignant ascites

Contact Info

Product

Resources

About