RESUMO

Primary effusion lymphoma (PEL) is an aggressive and rare type of diffuse large B-cell lymphoma (DLBL) that commonly presents itself as pleural, pericardial or peritoneal effusion without lymph node or extranodal involvement in immunosuppressed patients, such as HIV-positive or transplanted receptors. On rare occasions, it may be found in solid sites without effusion, in an immunophenotypically and morphologically similar neoplasm well-known as extracavitary PEL (EC-PEL). Both PEL and EC-PEL are associated with extremely poor prognosis. Due to the rarity of these entities, ther e are no gold standard treatments . Here we discuss the role of autologous bone marrow transplant (auto-BMT) in the treatment of these patients as well as report the case of a young HIV-positive male diagnosed with both PEL and EC-PEL, who underwent a salvage therapy with auto-BMT and achieved complete and sustained remission eight years after the diagnosis.

RESUMO

Primary effusion lymphoma (PEL) is an aggressive neoplasm often diagnosed in immunosuppressed patients demonstrating peritoneal, pleural, or pericardial effusions. This high-grade lymphoma is strongly associated with human herpesvirus 8 (HHV8) infection and most of the lesions also show the presence of Epstein-Barr virus in tumor cells, which lacks CD20 expression and reveals a plasmablastic morphology and phenotype. The extracavitary or solid variant of PEL is even rarer and usually affects the lymph nodes and is currently considered a clinical manifestation of the classic PEL. In the oral cavity, extracavitary PEL is extremely rare and only a few patients have been previously reported, with no detailed clinicopathological description. The recognition of oral extracavitary PEL is even more important given the occurrence of plasmablastic lymphoma in the oral mucosa, which shares many clinical, microscopic, and phenotypic features with PEL, therefore, demanding from pathologists the search for HHV8, especially in immunosuppressed patients, and an appropriate clinical evaluation. In this report, we aim to describe a very rare extracavitary PEL affecting the palate of a 36-year-old patient and to review the literature regarding the extracavitary presentation of this aggressive lymphoma. This report demonstrates the importance of searching for HHV8 infection in oral lymphomas with plasmablastic features.

Assuntos

RESUMO

Primary effusion lymphoma (PEL) is an aggressive B-cell lymphoma confined to body cavities and universally associated with human herpesvirus type 8 infection. The prognosis of this entity remains poor, with a median survival time of 6 to 9 months. To better understand the clinicopathologic features of the disease and identify possible prognostic factors, we performed a systematic review of the literature for cases of PEL, including 2 previously unreported cases from our institution. PEL was more prevalent in men (92%), with a median age at diagnosis of 55 years. The median overall survival for the entire series was 6 months. Peritoneal involvement (HR:1.62; 95% CI:1.06-2.48) and elevated serum lactate dehydrogenase (LDH) levels (HR:2.50; 95% CI:1.21-5.19) were associated with higher risk of death, while pericardial involvement (HR:0.43; 95% CI:0.20-0.94) was associated with lower risk of death. Therefore, effusion site and serum LDH levels are potential prognostic factors in patients with PEL.

Assuntos

RESUMO

BACKGROUND: Primary effusion lymphoma is a rare, high-grade non-Hodgkin's lymphoma that usually occurs in immunosuppressed or human immunodeficiency virus-positive individuals in advanced stages of the disease. However, primary effusion lymphoma occasionally affects immunocompetent patients who are infected with human herpes virus type 8 or Epstein-Barr virus. This disease manifests with liquid collections in cavities, producing constitutional symptoms; fever; weight loss; and symptoms related to extrinsic compression, such as dyspnea or abdominal discomfort. Diagnosis is confirmed with cytological or tissue evaluation showing large, multinucleated lymphoid cells with positive specific markers for the disease, such as CD45 and markers related to viral infections, when present. There is no standard treatment for primary effusion lymphoma, but several chemotherapy protocols are recommended, usually with poor results. CASE PRESENTATION: We present a case of an adult human immunodeficiency virus-negative Hispanic origin woman with primary effusion lymphoma with pleuritic, pericardial, and peritoneal compromise who also had unusual complications during a diagnostic procedure: the accidental rupture of the left ventricle and the development of a secondary left ventricular pseudoaneurysm. We describe the clinical, radiological, and laboratory characteristics as well as the outcome of this case. CONCLUSIONS: Primary effusion lymphoma is a very rare entity that represents 4% of non-Hodgkin's lymphoma cases associated with human immunodeficiency virus and 0.1% to 1% of all lymphomas in patients with another type of immunodeficiency in regions where human herpes virus type 8 is not endemic. This reported case is an unusual presentation of primary effusion lymphoma because it occurred in an immunocompetent human immunodeficiency virus-negative adult woman without the presence of Kaposi's sarcoma or Castleman's disease and for whom the clinical course after chemotherapy was successful. However, the rupture of the free wall of the left ventricle is a very rare catastrophic event that usually occurs after myocardial infarction. Left ventricle free wall rupture rarely goes unnoticed, but when it occurs, it leads to the development of a ventricular pseudoaneurysm in which the rupture is contained by the pericardium with an organized thrombus and an adjacent hematoma.

Assuntos