Case Of Primary Effusion Lymphoma Research Articles

A case of primary effusion lymphoma in an HIV-negative patient

背景：原発性体腔液リンパ腫（PEL）は，主に human immunodeficiency virus（HIV）感染患者に生じるまれな B 細胞性リンパ腫である．今回われわれは HIV 陰性患者に生じた PEL の 1 例を経験したので報告する．

A case of primary effusion lymphoma with the presence of human herpesvirus-8 DNA in a pleural effusion smear

A case of primary effusion lymphoma with the presence of human herpesvirus-8 DNA in a pleural effusion smear

Remission of an HHV8-related extracavitary primary effusion lymphoma in an HIV-positive patient during antiretroviral treatment containing dolutegravir

BackgroundHuman herpes virus 8 (HHV8) is the causative agent of Kaposi’s sarcoma and has been associated with an increasing number of hematologic diseases such as primary effusion lymphoma (PEL) (both classic and extracavitary form), multicentric Castleman disease and the germinotropic lymphoproliferative disorder. PEL is a rare B cell non-Hodgkin lymphoma that primarily affects immunocompromised patients; aggressive chemotherapy and antiretroviral therapy (ART) with protease inhibitors have been used, with poor results. We present a case of extracavitary PEL in an HIV-infected patient, regressed after ART initiation.Case presentationA 42-year-old male was admitted to the emergency room because of several months of malaise, fever and progressive deterioration of the general conditions. On physical examination soft non-painful subcutaneous masses were palpable at retronuchal, retroauricolar and thoracic regions. HIV serology resulted positive: HIV plasma viremia was 782,270 copies/mL, CD4 103 cells/mL. The excision of one of the masses, metabolically active at a positron emission tomography (PET-CT) scan, revealed an HHV8-related extracavitary PEL. HHV8 plasma viremia was 44,826 copies/mL. ART with tenofovir alafenamide/emtricitabine/dolutegravir was started together with ganciclovir for cytomegalovirus chorioretinitis. The progressive disappearance of the masses was seen after 6 weeks of ART, and a PET-CT scan resulted completely negative at 3 months. After 19 months of ART the patient was in remission of PEL, HIV viremia was undetectable (< 20 copies/mL), CD4 count was 766 cells/mL and HHV8 viremia was undetectable.ConclusionsIn this clinical case, the complete regression of PEL has been achieved after the immune recovery, as a consequence of ART introduction, without chemotherapy. It cannot be excluded that ganciclovir, used for the treatment of CMV chorioretinitis, may have contributed to the control of HHV8 replication. Whether to try or not a conservative approach in HIV-infected PEL patients must be carefully evaluated, considering the patient’s characteristics and the prognostic factors.

The utility of multiparametric flow cytometry in the detection of primary effusion lymphoma (PEL).

Primary effusion lymphoma (PEL) is a rare B cell lymphoproliferative disorder that arises predominantly in body cavities causing malignant effusions. The incidence of PEL is very low, accounting for approximately 4% of all HIV-associated non-Hodgkin lymphomas. PEL has also been described in elderly patients and after solid-organ transplantation. It is associated in all cases with human herpes virus 8 (HHV8). We describe a case of PEL in a 88-year-old HIV-negative woman who presented with progressive dyspnea and moderate right-sided pleural effusion without significant lymphadenopathies or other effusions. The cytological study of the pleural fluid revealed a dense proliferation of large plasmablastic cells. A six-color multiparametric flow cytometry immunophenotyping study was carried out, and revealed 45% of large in size and high cellular complexity cells positive for CD45 (dim), CD38, CD138, CD30 and HLA-DR; and negative for CD19, CD20, cytoplasmatic CD79a, surface and cytoplasmic light chains Kappa and Lambda, CD3, CD4, CD5, CD7, CD8, CD28, CD56, CD81, and CD117. In situ hybridization for EBV-encoded smalI RNA was negative and immunohistochemistry for Kaposi sarcoma herpesvirus (HHV8) confirmed the diagnosis of PEL. Our results confirm that flow cytometry bring useful data in the diagnosis of large-cell lymphomas involving body cavities. © 2018 International Clinical Cytometry Society.

A case of Epstein–Barr virus-negative human immunodeficiency virus-related primary effusion lymphoma

Primary effusion lymphoma (PEL) is a rare subtype of large B-cell lymphoma associated with human herpesvirus-8. Most cases are co-infected with Epstein-Barr virus (EBV). The prognosis of PEL is extremely poor and no optimal treatment regimen has been established. We report a case of EBV-negative PEL in a 49-year-old human immunodeficiency virus-positive man, presenting with massive bilateral pleural effusion.

Extracavity primary effusion lymphoma presenting in a lymph node without lymphomatous effusions

Primary effusion lymphoma (PEL) is a distinct clinicopathologic entity associated with human herpesvirus 8 (HHV8), which usually presents as a lymphomatous body cavity effusion. Rare cases of PEL are extracavitary and manifest as a solid tumor mass. Here we report an unusual case of extracavitary PEL (EPEL) involving lymph nodes with no evidence of a body cavity effusion. The neoplasm was comprised of large pleomorphic cells with prominent nucleoli and frequent mitotic figures that expressed HHV8, EBV, CD38 and CD30, but lacked all lineage-specific markers. The patient was found to be HIV positive after the diagnosis of EPEL. Antiretroviral therapy initially reduces tumor size, but the patient expired three months later due to multiple complications. PEL and EPEL are differing manifestations of a rare disease entity as defined by the World Health Organization 2008 classification. It is unknown why a small subset of these cases forms extracavitary masses with or without malignant effusions. Comparison with mouse xenograft models may provide unique insights into the pathogenesis of this disease for possible future studies.

CLINICAL AND VIROLOGICAL CHARACTERISTICS OF DISEASES ASSOCIATED WITH THE KAPOSI SARCOMA VIRUS

Abstract. The paper presents literature data and results of own studies on the Kaposi's sarcoma herpesvirus (KSHV) and its association with Kaposi's sarcoma (KS), the primary effusion lymphoma (PEL) and multicentric Kastleman disease (MCD). The obtained data show that all 3 main clinical forms of KS, diagnosed in Russia, are significantly associated with KSHV, though not always in 100% of cases. Distinct groups of persons with high prevalence of virus infection have been detected among patients with other forms of cancer and non-cancer diseases that may represent a risk factor for development of KS. It was found that the prostate, especially in patients with carcinoma of the prostate and KS could be an important localization of virus and intimate contact with these patients should be limited. For the first time in Russia HIV-negative case of PEL associated with KSHV had been diagnosed and studied in details. It was also demonstrated that isolates of KSHV, circulating in the country, belong to the two major genetic subgroups of the virus (A and C), a widely represented in the European countries and in the United States, which indicates a common origin of the virus in these countries. The current methodologies of KS’ treatment are also presented.

A case of primary effusion lymphoma diagnosed by open cardiocentesis

Primary effusion lymphoma is a type of B-cell lymphoma that is primarily related to human immunodeficiency virus. Thoracic surgeons rarely encounter this disease because of its rarity. We herein report a case of primary effusion lymphoma which required surgery for successful treatment. An 83-year-old man was admitted to our hospital with signs of cardiac tamponade. A radiological examination revealed a pericardial effusion. Performing percutaneous pericardiocentesis was difficult due to the patient's anatomical features. We performed open cardiocentesis under general anesthesia. A cytologic examination revealed primary effusion lymphoma. The patient underwent chemotherapy, resulting in complete remission.

Castleman’s disease and primary effusion lymphoma in a HIV-positive patient

We present a case of primary effusion lymphoma (PEL) occurring simultaneously with Castleman's disease in the same patient. Castleman's disease is distinct from PEL, although both are associated with HHV-8. Other cases have debated whether the coexistence of PEL and Castleman's disease is a recurrence of an original PEL tumour in an extracavitary site, or a secondary HHV-8-associated lymphoma distinct from primary PEL. Our case, along with those described previously, show that co-occurrence of PEL and Castleman's disease is possible and plausible. PEL needs to be included in the differential diagnosis in any HIV-positive patient who presents with a pleural effusion, and diagnosis requires only a simple thoracentesis and appropriate immunohistochemistry.

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.

A Case of Primary Effusion Lymphoma in a Patient with Chronic Kidney Disease

Human herpes virus-8 (HHV-8)-negative primary effusion lymphoma (PEL) is frequently observed in patients with chronic liver disease. We describe herein a case of PEL in a patient with chronic kidney disease. A 59-year-old woman presented with dyspnea. The patient had a history of uncontrolled pleural effusion. The pleural effusion revealed a malignant cell-dominant exudate. Serological tests were negative for human immunodeficiency virus (HIV). Initial chest and abdomen computed tomography showed bilateral pleural effusion; however, no evidence of a tumor mass or lymph node enlargement was found. A malignant lymphoma of diffuse large B-cell type was confirmed by pleural fluid cytology. Immunohistochemical staining of malignant cells was negative for HHV-8. She was treated with rituximab and CHOP (cyclophosphamide, adriamycin, vincristine, prednisolone) chemotherapy. We report a case of PEL in a patient with chronic kidney disease that may be a plausible predisposing factor for HHV-8-negative PEL. (Korean J Med 2012;83:385-389)

Primary Effusion Lymphoma/Extracavitary Effusion Lymphoma

Primary effusion lymphoma (PEL), and its solid counterpart, extracavitary PEL, is a rare, but unique, clinicopathologic entity, which is pathogenetically related to infection with the gamma herpesvirus, Kaposi sarcoma herpesvirus/human herpes virus 8. Although it occurs primarily in individuals positive for human immunodeficiency virus infection with low CD4 counts, it can develop in other patient populations as well. The tumor cells, which are morphologically pleomorphic, are usually positive for Epstein-Barr virus infection, classically lack expression of B- and T-cell–associated antigens, and are often positive for activation antigens such as CD30 and epithelial membrane antigen. Molecular studies show that they are of B-cell origin but usually lack structural alterations in oncogenes and tumor suppressor genes such as MYC. However, without attention to clinical history, careful examination of the cytomorphology and investigation for the presence of Kaposi sarcoma herpesvirus/human herpes virus 8, PEL/EC-PEL may be misdiagnosed or unrecognized. Here a case of PEL occurring in a patient positive for human immunodeficiency virus infection is described with a discussion of the pertinent cliniopathologic features of this unique entity.

Primary effusion lymphoma involving the cerebrospinal fluid

Primary effusion lymphoma or body cavity based lymphoma is a form of large B-cell lymphoma which usually presents as serous effusions without detectable tumor masses. It is universally associated with human herpesvirus-8 also known as Kaposi sarcoma herpesvirus. This condition, so far, has been reported in the body cavity effusions that include pleura, peritoneum, and pericardium. We report a case of primary effusion lymphoma which has involved the cerebrospinal fluid. To our knowledge, this is the first case of PEL reported in the cerebrospinal fluid.

Primary effusion lymphoma involving three body cavities

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

Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia

Although primary effusion lymphoma (PEL) is usually associated with human herpes virus-8/Kaposi sarcoma herpes virus (HHV-8/KSHV) and human immunodeficiency virus (HIV), there are several reports of HHV-8/KSHV and HIV negative cases, mainly in the setting of immunodeficiency. Here, we report the second case of PEL associated with idiopathic T4 lymphocytopenia (ICL), which was HHV-8/KSHV negative, HIV negative and Epstein-Barr virus positive, while no other causative agents for immunodeficiency were documented. Flow cytometry revealed a hyperdiploid and highly mitotic large B-cell population, CD30, EMA, CD66, CD38 and CD71 positive. The malignant lymphoma cells showed atypia with prominent nuclei and basophilic vacuolated cytoplasm, while cytogenetic analysis with fluorescent in situ hybridization showed trisomy 18. The patient was administered R-COP chemotherapy, but no remission was achieved, up to 3 months from diagnosis.

A case of primary effusion lymphoma that originated from the pericardium

A case of primary effusion lymphoma that originated from the pericardium

Primary effusion lymphoma with cardiac involvement in HIV positive patient—Complete response and long survival with chemotherapy and HAART

Primary effusion lymphoma (PEL) is a rare type of lymphoma related to herpesvirus-8 (HHV-8), and considered an AIDS-defining condition. The authors describe a case of PEL with cardiac involvement occurring in an HIV-positive patient treated with HAART and chemotherapy, who achieved complete remission and long survival.

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

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 hyperchromatic 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.

Primary effusion lymphoma of the pericardial cavity carrying t(1;22)(q21;q11) and t(14;17)(q32;q23)

We report a case of primary effusion lymphoma (PEL) in a 75-year-old woman without human immunodeficiency virus or hepatitis C virus, which presented as fever, chest pain, and pericardial effusion. The lymphoma cells were positive for CD20 and CD79a, and were negative for CD3 and CD10. Genomic human herpes virus 8 (HHV-8) and Epstein-Barr virus were not detected in the lymphoma cells. Cytogenetic analysis showed complex abnormalities by the G-banding technique, and spectral karyotyping (SKY) analysis provided more detailed characterization of the chromosomal aberrations, including t(1;22)(q21;q11) and t(14;17)(q32;q23). We did not detect C-MYC gene rearrangement or BCL-2 expression. She was treated successfully with six courses of the CHOP regimen. The present case demonstrated a rare category of PEL that is not associated with HHV-8 or C-MYC gene rearrangement. In addition, SKY analysis disclosed cryptic chromosomal abnormalities involving 1q21 and 17q23.

Primary effusion lymphoma with B-cell phenotype

We describe here a case of primary effusion lymphoma that occurred in a 78-year-old woman. She was successively treated with prednisolone but died 15 months after the diagnosis of primary effusion lymphoma. The immunohistochemistry revealed the neoplastic cells to be CD19(+), CD20(+), CD21(+), Sm-Ig(+), and HLA-DR(+). This patient exhibited clonal IgH and clonal kappa light chain gene rearrangement, indicating a B-cell origin. The present case was distinguished from the majority cases of HHV-8-positive primary effusion lymphoma. Here we present clinical details of response to therapy in this case.