An unusual case of Primary Effusion Lymphoma with aberrant T-cell phenotype in a HIV-negative, HBV-positive, cirrhotic patient, and review of the literature

Primary effusion lymphoma (PEL) is an unusual, human herpes virus-8 (HHV-8)–associated type of lymphoma, presenting as lymphomatous effusion in body cavities, without a detectable tumor mass. It primarily affects human immunodeficiency virus (HIV)-infected patients, but has also been described in other immunocompromised individuals. Although PEL is a B-cell lymphoma, the neoplastic cells are usually of the ‘null’ phenotype by immunocytochemistry. This report describes a case of PEL with T-cell phenotype in a HIV-negative patient and reviews all the relevant cases published until now. Our patient suffered from cirrhosis associated with Hepatitis B virus (HBV) infection and presented with a large ascitic effusion, in the absence of peripheral lymphadenopathy or solid mass within either the abdomen or the thorax. Paracentesis disclosed large lymphoma cells with anaplastic features consisting of moderate cytoplasm and single or occasionally multiple irregular nuclei with single or multiple prominent nucleoli. Immunocytochemically, these cells were negative for both CD3 and CD20, but showed a positive reaction for T-cell markers CD43 and CD45RO (VCHL-1). Furthermore, the neoplastic cells revealed strong positivity for EMA and CD30, but they lacked expression of ALK-1, TIA-1, and Perforin. The immune status for both HHV-8 and Epstein-Barr virus (EBV) was evaluated and showed positive immunostaining only for the former. The combination of the immunohistochemistry results with the existence of a clonal rearrangement in the immunoglobulin heavy chain gene (identified by PCR), were compatible with the diagnosis of PEL. The presence of T-cell markers was consistent with the diagnosis of PEL with an aberrant T-cell phenotype.

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T-cell Primary Effusion Lymphoma in an Elderly HIV-Negative Patient Coinfected With Human Kaposi Sarcoma Virus and Epstein-Barr Virus

American Journal of Clinical Pathology ◽

10.1093/ajcp/144.suppl2.156 ◽

2015 ◽

Vol 144 (suppl 2) ◽

pp. A156-A156

Author(s):

Melanie Kubik ◽

John Thorson ◽

Huan-You Wang

Keyword(s):

T Cell ◽

Epstein Barr Virus ◽

Primary Effusion Lymphoma ◽

Kaposi Sarcoma ◽

Negative Patient ◽

Barr Virus ◽

Sarcoma Virus ◽

Epstein Barr ◽

Hiv Negative

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An unusual case of posttransplant peritoneal primary effusion lymphoma with T-cell phenotype in a HIV-negative female, not associated with HHV-8

Pathology & Oncology Research ◽

10.1007/bf02893396 ◽

2005 ◽

Vol 11 (3) ◽

pp. 178-181 ◽

Cited By ~ 19

Author(s):

Ioannis Venizelos ◽

Demetrio Tamiolakis ◽

Maria Lambropoulou ◽

Sylva Nikolaidou ◽

Sophia Bolioti ◽

...

Keyword(s):

T Cell ◽

Unusual Case ◽

Primary Effusion Lymphoma ◽

Cell Phenotype ◽

Hiv Negative

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Primary effusion lymphoma involving three body cavities

CytoJournal ◽

10.4103/1742-6413.56361 ◽

2009 ◽

Vol 6 ◽

pp. 21 ◽

Cited By ~ 15

Author(s):

Fadi Brimo ◽

Gizelle Popradi ◽

René P Michel ◽

Manon Auger

Keyword(s):

Primary Effusion Lymphoma ◽

B Cell Lymphoma ◽

Large Cell ◽

Immunocompetent Patient ◽

Nuclear Antigen ◽

Positive Staining ◽

Neoplastic Cells ◽

Current Case ◽

Human Herpes Virus 8 ◽

Three Body

Primary effusion lymphoma (PEL) is a human herpes virus-8 (HHV8)-associated large-cell non-Hodgkin lymphoma localized in body cavities and presenting as pleural, peritoneal, or pericardial lymphomatous effusions. It typically affects immunocompromised patients and usually involves only one body site. We describe herein a case of PEL affecting three body cavity sites in an immunocompetent patient. A 69-year-old HIV-negative man presented with upper gastrointestinal bleeding and ascites. An examination of the fluid by cytology showed large atypical lymphocytes with abundant basophilic cytoplasm, either central or eccentric nuclei having irregular outlines, and multiple prominent nucleoli. The neoplastic cells showed positive staining for CD45, CD3, HHV8 latent nuclear antigen (LNA), and Epstein-Barr virus-encoded RNA. A diagnosis of PEL was rendered. Despite chemotherapy and valganciclovir, the disease progressed to involve the pleural and pericardial cavities and the patient died 5 months following the initial diagnosis. Although PEL is a B-cell lymphoma, it is usually of null phenotype by immunohistochemistry, and can rarely aberrantly express T-cell markers, as seen in the current case. The key to the diagnosis of PEL rests on identifying HHV8 in the neoplastic cells. Therefore, restricting the term of PEL only to those cases that are HHV8 positive is important in order to differentiate PEL from other lymphomas that can present as serous effusions and that carry, in general, a more favorable prognosis than PEL

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High-Grade Epstein-Barr Virus-Negative Biphenotypic Lymphoma with Expression of B- and T-Cell Markers and Leukemia Presentation: Case Report and Literature Review

Case Reports in Oncology ◽

10.1159/000510403 ◽

2020 ◽

Vol 13 (3) ◽

pp. 1215-1226

Author(s):

Samah Kohla ◽

Feryal A. Ibrahim ◽

Deena Mudawi ◽

Susanna Akiki ◽

Dina Soliman ◽

...

Keyword(s):

T Cell ◽

B Cell ◽

Epstein Barr Virus ◽

B Cell Lymphoma ◽

High Grade ◽

Aberrant Expression ◽

Cell Markers ◽

Cell Antigen ◽

Barr Virus ◽

Epstein Barr

Lymphomas are presently categorized according to their origin from B or T lymphocytes. The co-expression of CD3 in B-cell lymphomas or CD20 in T-cell lymphomas has been rarely reported. Immature and less often mature lymphomas may incorporate the rearrangements of both B- and T-cell antigen receptor genes (dual genotype or bigenotype). Lymphoma cells with a sole genotype hardly concurrently express both B- and T-cell markers (biphenotypic lymphomas). We describe a 63-year-old female who was presented with obstructive jaundice and epigastric pain of 10 days. Initial CBC revealed 43x10<sup>3</sup>/μL white blood cells, 11.2 g/dL hemoglobin, and 88x10<sup>3</sup>/μL platelets. CT abdomen revealed hepatomegaly and suspected pancreatic mass with large retroperitoneal lymph nodal mass. Peripheral smear showed 56% lymphoid cells with blast morphology. The bone marrow (BM) aspirate smear was infiltrated by 83% immature-looking cells. BM biopsy showed interstitial to diffuse extensive infiltration by primitive-looking cells, positive for pan-B-cell antigens CD20, CD79, and PAX5 as well as the T-cell antigen CD4, CD5, CD3, while negative for all immaturity markers (CD34, TdT, and CD1a). In situ hybridization for Epstein-Barr virus (EBV)-encoded small RNA (EBER) was negative. Flow cytometry on BM aspirate showed an abnormal population (50%) expressing the B-cell antigens (CD19, CD20, CD79, CD22) and CD10, and showed lambda light chain restriction as well as the T-cell antigens cCD3 and CD4 with partial CD5. The analysis showed, also, another abnormal population of lambda restricted monotypic B cells (8%) with dimmer CD45 (blast gate) and showed the same immunophenotype (expressing the B-cell antigens), but negative for CD10, cCD3, CD5, and CD4. Conventional cytogenetic revealed complex karyotype. Molecular studies revealed rearrangements of the immunoglobulin heavy chain region consistent with a clonal B-cell population. TCR gene rearrangement analysis was equivocal concerning clonality but was not conclusive for clonal T-cell disease. Our final diagnosis was peripheral blood and BM involvement by EBV-negative high-grade lymphoid neoplasm (in leukemic phase with blast morphology) and an ambiguous immunophenotype with a differential diagnosis that may include the rare entity of bigenotypic lymphoma or an unusual case of high-grade B-cell lymphoma with aberrant expression of T-cell markers (biphenotypic lymphomas).

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Epidemiology of Non-Hodgkin’s Lymphoma

Medical Sciences ◽

10.3390/medsci9010005 ◽

2021 ◽

Vol 9 (1) ◽

pp. 5

Author(s):

Krishna C. Thandra ◽

Adam Barsouk ◽

Kalyan Saginala ◽

Sandeep Anand Padala ◽

Alexander Barsouk ◽

...

Keyword(s):

T Cell ◽

Radiation Treatment ◽

Cell Lymphoma ◽

Breast Implants ◽

Occupational Exposures ◽

B Cell Lymphoma ◽

Large Cell ◽

T Cell Lymphoma ◽

Human Herpes Virus 8 ◽

Human T Cell

Non-Hodgins’s lymphoma (NHL) is the most common hematological malignancy worldwide, accounting for nearly 3% of cancer diagnoses and deaths. NHL is the seventh most prevalent cancer and has the sixth highest mortality among cancers in the US. NHL accounts for 4% of US cancer diagnoses, and incidence has increased 168% since 1975 (while survival has improved 158%). NHL is more common among men, those >65 years old, and those with autoimmune disease or a family history of hematological malignancies. NHL is a heterogenous disease, with each subtype associated with different risk factors. Marginal zone lymphoma (MZL) is strongly associated with Sjogren’s syndrome (SS) and Hashimoto’s thyroiditis, while peripheral T-cell lymphoma (PTCL) is most associated with celiac disease. Occupational exposures among farm workers or painters increases the risk of most of the common subtypes. Prior radiation treatment, obesity, and smoking are most highly associated with diffuse large B-cell lymphoma (DLBCL), while breast implants have been rarely associated with anaplastic large cell lymphoma (ALCL). Infection with Epstein–Barr Virus (EBV) is strongly associated with endemic Burkitts lymphoma. HIV and human herpes virus 8 (HHV-8), is predisposed to several subtypes of DLBCL, and human T-cell lymphoma virus (HTLV-1) is a causative agent of T-cell lymphomas. Obesity and vitamin D deficiency worsen NHL survival. Atopic diseases and alcohol consumption seem to be protective against NHL.

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A Case of Cutaneous Epstein-Barr Virus-Associated Diffuse Large B-Cell Lymphoma in an Angioimmunoblastic T-Cell Lymphoma

Annals of Dermatology ◽

10.5021/ad.2016.28.6.789 ◽

2016 ◽

Vol 28 (6) ◽

pp. 789 ◽

Cited By ~ 1

Author(s):

Mi-Hye Lee ◽

Ik-Jun Moon ◽

Woo-Jin Lee ◽

Chong-Hyun Won ◽

Sung-Eun Chang ◽

...

Keyword(s):

T Cell ◽

B Cell ◽

Epstein Barr Virus ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Angioimmunoblastic T Cell Lymphoma ◽

Barr Virus ◽

Large B Cell Lymphoma ◽

Epstein Barr ◽

Large B Cell

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Lymphomas in Patients With Sjögren's Syndrome Are Marginal Zone B-Cell Neoplasms, Arise in Diverse Extranodal and Nodal Sites, and Are Not Associated With Viruses

Blood ◽

10.1182/blood.v90.2.766 ◽

1997 ◽

Vol 90 (2) ◽

pp. 766-775 ◽

Cited By ~ 241

Author(s):

Bruno Royer ◽

Dominique Cazals-Hatem ◽

Jean Sibilia ◽

Felix Agbalika ◽

Jean-Michel Cayuela ◽

...

Keyword(s):

B Cell ◽

Marginal Zone ◽

P53 Protein ◽

B Cell Lymphoma ◽

Lymphoid Tissues ◽

Low Grade ◽

Human Herpes Virus 8 ◽

Barr Virus ◽

P53 Antibodies ◽

Histological Transformation

Abstract The occurrence of non-Hodgkin's lymphoma (NHL) is the most serious complication of Sjögren's syndrome (SS). We performed a study of 16 NHLs occurring in patients with an underlying SS. These lymphomas arose not only in salivary glands (7 cases) but also in other mucosal extranodal sites (the stomach [4 cases], the lung [3 cases], the skin [3 cases], the buccal mucosa [1 case], the thymus [1 case]) and in nodal sites (8 cases). Low-grade marginal zone lymphomas (MZL) were diagnosed in 12 of the 16 patients, 9 of mucosa-associated lymphoid tissues (MALT) type in mucosal sites and 3 exclusively nodal. The 4 other patients presented with a high-grade B-cell lymphoma that was probably a histological transformation of an underlying low-grade MZL at least in 3 of the cases involving skin, stomach, and parotid, respectively. A t(14; 18) translocation was detected in 1 of 8 lymphomas tested. We detected serum anti-p53 antibodies in 2 of the 14 studied patients. p53 protein was detected in 1 of 11 lymphomas tested. LMP protein and Eber RNAs of Epstein-Barr virus (EBV) were not detected in the 16 NHL biopsies. Using polymerase chain reaction, EBV was never detected except in 1 of 4 parotid lymphomas. No human T-lymphotropic virus 1 or human herpes virus 8 DNAs were detected in NHL biopsies. None of the patients had hepatitis C virus infection found using serological methods. Chemotherapy was usually efficient. In conclusion, lymphomas occurring in patients with an underlying SS are in most cases MZL. These lymphomas are not associated with viruses known to be present in other types of lymphomas. Some of the translocations or mutations of oncogenes or antioncogenes described in other lymphomas are detected in SS-associated lymphomas.

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Multiple remissions of extracavitary primary effusion lymphoma treated with a single cycle of liposomal doxorubicin in a patient infected with HIV

Current Oncology ◽

10.3747/co.25.4119 ◽

2018 ◽

Vol 25 (6) ◽

Cited By ~ 4

Author(s):

J. Chen ◽

V. Mehraj ◽

J. Szabo ◽

B. Routy ◽

R. P. Michel ◽

...

Keyword(s):

T Cell ◽

Complete Remission ◽

Hiv Infection ◽

Liposomal Doxorubicin ◽

Primary Effusion Lymphoma ◽

Human Herpesvirus ◽

B Cell Lymphoma ◽

Cd4 T Cell ◽

Cell Recovery ◽

Single Cycle

Primary effusion lymphoma (pel) is a rare human herpesvirus 8 (hhv8)–related large B cell lymphoma with plasmablastic, immunoblastic, or anaplastic features that often carries a poor prognosis. This lymphoma occurs mainly in patients with hiv infection, most often with Epstein–Barr virus (ebv) co-infection, and usually presents as body cavity effusions or, less commonly, as extracavitary lesions without effusion (ec-pel). Chemotherapeutic treatment options are limited and require concurrent antiretroviral therapy (art).Here, we report the case of an adult patient with hiv infection and chronic hepatitis E virus (hev) co-infection who had low CD4 T cell recovery after years of art. The patient then developed a cutaneous ec-pel which rapidly regressed after 1 cycle of liposomal doxorubicin (ld) for his Kaposi sarcoma (ks) before treatment with chop chemotherapy. He had previously received numerous cycles of ld for cutaneous ks over 2 years.Because of the patient’s low CD4 T cell count, hev co-infection, and earlier unexpected remission of ec-pel before chop, the patient opted for a single trial of ld before other options. Surprisingly, he experienced a complete remission lasting 18 months. Subsequently, his ec-pel relapsed twice at 31 and at 41 months after the initial diagnosis. Upon recurrence, a similar single cycle of ld was given, which again induced remission. The patient today is in complete remission after a total of 4 ld infusions over 54 months.This patient represents a unique case of hiv-with-hhv8–related, ebv-negative ec-pel with chronic hev coinfection, in which rapid remission was achieved after a single cycle of ld, suggesting an antiviral response in addition to the chemotherapeutic effect.

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