Primary effusion lymphoma in human immune deficiency (HIV)‐negative non‐organ transplant immunocompetent patients

Yuan, Lisi; Cook, James R.; Elsheikh, Tarik M.

doi:10.1002/dc.24371

Cited by 6 publications

(6 citation statements)

References 41 publications

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“…These data provide an interesting perspective to age-related changes in immune function that may be a risk factor for active HHV8 infection, despite the absence of HIV [19]. However, in other studies of HIV-negative PEL, CD4 count was not reported [10,20,21].…”

Section: The Association Of Immunodeficiency With Pelmentioning

confidence: 77%

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Primary Effusion Lymphoma: A Timely Review on the Association with HIV, HHV8, and EBV

2022

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Primary effusion lymphoma (PEL) is defined by the WHO classification as a large B-cell neoplasm without detectable tumor masses. It is universally associated with HHV8, with most cases occurring in the setting of immunodeficiency such as HIV infection, and a poor prognosis. Morphologically, the neoplastic cells range from immunoblastic, plasmablastic, to anaplastic; and phenotypically, most cases express plasma cell but not B-cell markers, i.e., plasmablastic. During the past decade, primary HHV8-negative effusion lymphoma has been reported. Such cases were considered in the WHO classification scheme as effusion-based lymphoma. We performed a systemic review of 167 HHV8-negative effusion lymphomas from the literature and found that only 42% were associated with a fluid overload state, and with low rates of HIV (6%) or EBV (21%) infection. Furthermore, most patients are old (or immunosenescent) with underlying medical conditions/comorbidities, most neoplasms are of B-cell phenotype, and the outcome is more favorable than that of HHV8-positive PEL. These distinctive findings supported our prior proposal of designating these HHV8-negative cases as type II PEL, in contrast to the classic or type I PEL as defined by the WHO. Furthermore, we propose an algorithmic approach for the diagnosis of PEL and its mimickers.

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Section: The Association Of Immunodeficiency With Pelmentioning

confidence: 77%

“…PEL has been reported in HIV and HHV8-negative elderly patients without immunodeficiency [10,19,20,38]. In our previous study of 26 Taiwanese patients with a median age of 76.5, only one case was HIV-positive, while the others were either negative or presumed negative based on the clinical setting [10].…”

Section: Immunosenescencementioning

confidence: 92%

Primary Effusion Lymphoma: A Timely Review on the Association with HIV, HHV8, and EBV

2022

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“…The tumor cells were positive for CD45, CD30, MUM1, and HHV8 and negative for HMB45, CD3, CD20, PAX5, ALK1, and CD138 confirming diagnosis of PEL. Also in their report, they found other 27 cases of PEL in HIV negative individuals reported in the literature, majority being males in their 70s with 32% from a Mediterranean origin 7 …”

Section: Discussionmentioning

confidence: 76%

“…Also in their report, they found other 27 cases of PEL in HIV negative individuals reported in the literature, majority being males in their 70s with 32% from a Mediterranean origin. 7 The diagnosis of PEL may be challenging since it is based on the combination of clinical symptoms, morphology and immunophenotype, and molecular findings. However, the cytological assessment of the fluid or biopsy of the compromised membrane remain the mainstay for diagnosis because it typically lacks a solid mass.…”

Section: Case Reportmentioning

confidence: 99%

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

Malik

Khader

Asiry

2021

Diagnostic Cytopathology

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We present a rare case of primary effusion lymphoma (PEL) in a 75 year old, HIV‐negative male patient with multiple comorbidities. Imaging studies revealed a massive right pleural effusion and a significant lung collapse with multiple plural soft tissue nodules. Immediate thoracentesis was performed. Cytologic evaluation of the pleural fluid showed abnormally large cell with increased nuclear to cytoplasmic ratio, irregular nuclear contours and prominent nucleoli, with phenotypic expression of HHV‐8, CD138, CD30, and MUM1 markers and negative staining for epithelial and mesothelial markers. PEL is a rare and aggressive large B‐cell lymphoma often affecting immunocompromised adults and is mostly associated with human herpes virus 8/Kaposi sarcoma‐associated herpes virus (HHV‐8/KSHV). However, cases in immunocompetent elderly patients have been reported. The cytomorphologic features of PEL overlaps with those of aggressive lymphomas such as diffuse large B‐cell lymphoma, plasmablastic lymphoma, and anaplastic large‐cell lymphoma. Also, mesothelioma, metastatic carcinoma or melanoma should be considered in the differential diagnosis. Hence, PEL should be kept in mind in the diagnostic algorithm of cytological evaluation of serosal fluid not only in HIV positive patients but also HIV‐negative elderly patients. In this report, we aim to highlight the cytologic and immunohistochemical staining pattern of this rare entity to increase awareness of this entity among cytopathologists.

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“…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.…”

Section: Patient Demographics Comorbidities and Pel Sitementioning

confidence: 99%

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

Cozzi¹,

Rossi²,

Rullo³

et al. 2022

Mediterr J Hematol Infect Dis

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Primary effusion lymphoma (PEL) is a large B-cell lymphoma growing within body-cavities caused by the Kaposi sarcoma-associated herpesvirus (KSHV)/human herpesvirus-8 (KSHV/HHV-8). It is mainly reported in HIV-infected patients. The uncommon occurrence in the elderly supports a form paralleling classic Kaposi sarcoma (KS), i.e. classic PEL, whose characteristics are relatively underexplored. To better understand the diagnostic modalities and clinical-epidemiological features of classic PEL, articles reporting cases of PEL were identified through MEDLINE/EMBASE databases (January 1998-July 2020) and screened according to PRISMA guidelines to extract individual-level data. A comparison was also performed between classic PEL and classic KS to evaluate similarities and differences. We identified 105 subjects (median age 77 years; 86% males), mainly from Mediterranean countries (52%, first Italy) and Eastern Europe (7%). Common comorbidities were heart failure (32%), cirrhosis (16%), and malignancy (20%) including lymphoid neoplasms. Pleural cavity was the commonest site (67%). PEL diagnosis was based on cytomorphology (89%), evidence of KSHV/HHV-8 infection (94%), EBV co-infection (28%) and clonality of IGH (59%), IGK (14%), TRG (9%) alone or in multiple combinations. Compared to KS, age (P<.001), gender-ratio (P=.08) and mortality (P<.001) were significantly higher in PEL, whereas the frequency of PEL as a second primary was similar (P=.44). This is the first systematic review of classic PEL case reports highlighting heterogeneity and lack of a uniform multidisciplinary approach at diagnosis, in the absence of specific guidelines as it happens for rare cancers. It is conceivable that classic PEL is still underdiagnosed in Mediterranean countries wherein KSHV/HHV-8 is endemic.

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Primary effusion lymphoma in human immune deficiency (HIV)‐negative non‐organ transplant immunocompetent patients

Cited by 6 publications

References 41 publications

Primary Effusion Lymphoma: A Timely Review on the Association with HIV, HHV8, and EBV

Primary Effusion Lymphoma: A Timely Review on the Association with HIV, HHV8, and EBV

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

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

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