Primary effusion lymphoma in a human immunodeficiency virus-negative patient with unexpected unusual complications: a case report

Abstract

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.

Classic KSHV/HHV-8-Positive Primary Effusion Lymphoma (Pel): A Systematic Review and Meta-Analysis of Case Reports

“…We included 74 studies in the final analysis, accounting for 105 individual patient cases. Over the 23-year period of publications, 32 studies were published during the first 12 years (1995–2008) 3 , 6 , 10 – 39 and 42 during the second 11 years (2009–2020) 40 – 81 as full articles (n=58), letters (n=7), images (n=5), and conference papers/abstracts (n=4). Sixty-eight (91.9%) articles reported one or two cases.…”

“…Demographics for 105 patients and clinical characteristics are listed in Table 1. The median (interquartile range, IQR) age was 77 (69)(70)(71)(72)(73)(74)(75)(76)(77)(78)(79)(80)(81)(82)(83)(84)(85) with no gender difference (P>.05). The male-to-female ratio was 6:1.…”

A rare case of primary effusion lymphoma in HIV negative patient: Diagnostic challenges and literature review

“…The typical cytological features of PEL are large, multinucleated or multilobed lymphoid cells with prominent nucleoli, basophilic cytoplasm with clear small vacuoles, and high mitotic activity. Immunophenotypically malignant cells express CD45+ and are negative for B‐cell markers (CD19, CD20, and CD79a) and T‐cell markers (CD3, CD4, CD8), respectively 1 …”

“…Immunophenotypically malignant cells express CD45+ and are negative for B-cell markers (CD19, CD20, and CD79a) and Tcell markers (CD3, CD4, CD8), respectively. 1 Molecular studies have reported PEL cell of origin is derived from postgerminal center B-cells approaching plasma cell differentiation. 5,9 This explains the frequent expression of activation markers like CD30 and plasma cell markers such as CD38, CD138, and EMA in PEL.…”

“…Primary effusion lymphoma (PEL) is a rare and aggressive high-grade distinct B cell non-Hodgkin lymphoma (NHL) with unique presentation as effusions in body cavities, such as the pleural space, pericardium, and peritoneum, without detectable tumor masses. 1 It is commonly associated with human herpes virus 8/Kaposi sarcomaassociated herpes virus (HHV-8/KSHV). 2 In 2001, PEL was first recognized and officially introduced as a distinct malignancy in the WHO classification of hematopoietic and lymphoid tissues neoplasms.…”