Langerhans cell histiocytosis and its relationship with Epstein-Barr virus

Schenka, André Almeida; Andrade, Liliana Aparecida Lucci De Angelo; Amstalden, Eliane Maria Ingrid; Cintra, Maria Letícia; Vassallo, José; Cardinalli, Izilda Aparecida; Azevedo, Amílcar Cardoso de; Brandalise, Sílvia Regina; Soares, Fernando Augusto

doi:10.1016/j.humpath.2006.07.008

Cited by 8 publications

(7 citation statements)

References 5 publications

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“…On the other hand, our findings of a low incidence in Blacks are not fully supported by an infectious theory, as literature also reports low vaccination coverage for black children, although disparities between whites non‐Hispanic and blacks non‐Hispanic have narrowed in the period of 2001–2010 . It is important to clarify that although two studies have demonstrated associations between infections, lack of immunizations, and LCH , other studies failed to show a possible viral etiology for the disease .…”

Section: Discussioncontrasting

confidence: 80%

Ethnicity, race, and socioeconomic status influence incidence of langerhans cell histiocytosis

Ribeiro

Degar

Antoneli

et al. 2015

Pediatric Blood & Cancer

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Section: Discussioncontrasting

confidence: 80%

Ethnicity, race, and socioeconomic status influence incidence of langerhans cell histiocytosis

Ribeiro

Degar

Antoneli

et al. 2015

Pediatric Blood & Cancer

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“…We think that our results therefore resolve the much debated issue of the role of EBV in the pathogenesis of LCH [19], [21], [22], [30], [31]. Because EBV infected B cells are present in the blood of healthy seropositive individuals [40] at a low frequency in the order of 10- 6 [41] it is not unexpected to find a small number of EBV infected B cells in LCH granuloma, which contains B cells.…”

Section: Discussionsupporting

confidence: 55%

“…However, since HHV-6 and EBV are responsible for persistent infection of T cells and B cells respectively, the detection of virus DNA in LCH samples could be attributed to infection of bystander cells [21].…”

Section: Resultsmentioning

confidence: 99%

“…Infection with EBV has been reported to be associated with LCH, to represent a possible etiology, and/or to contribute to its pathophysiology in some studies [11], [12], [19]. However, other studies failed to replicate these findings, and the possible causative role of EBV in LCH is debated [20], [21], [22]. CMV can also infect DC and LC [23], [24], [25], and one single study reported CMV detection in lesional LC in one third of 29 patients by immunohistochemistry, in situ hybridization, and PCR [26].…”

Section: Introductionmentioning

confidence: 99%

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Herpes-Virus Infection in Patients with Langerhans Cell Histiocytosis: A Case-Controlled Sero-Epidemiological Study, and In Situ Analysis

et al. 2008

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BackgroundLangerhans cell histiocytosis (LCH) is a rare disease that affects mainly young children, and which features granulomas containing Langerhans-type dendritic cells. The role of several human herpesviruses (HHV) in the pathogenesis of LCH was suggested by numerous reports but remains debated. Epstein-barr virus (EBV, HHV-4), & Cytomegalovirus (CMV, HHV-5) can infect Langerhans cells, and EBV, CMV and HHV-6 have been proposed to be associated with LCH based on the detection of these viruses in clinical samples.MethodologyWe have investigated the prevalence of EBV, CMV and HHV-6 infection, the characters of antibody response and the plasma viral load in a cohort of 83 patients and 236 age-matched controls, and the presence and cellular localization of the viruses in LCH tissue samples from 19 patients.Principal FindingsThe results show that prevalence, serological titers, and viral load for EBV, CMV and HHV-6 did not differ between patients and controls. EBV was found by PCR in tumoral sample from 3/19 patients, however, EBV small RNAs EBERs –when positive-, were detected by in situ double staining in bystander B CD20+ CD79a+ lymphocytes and not in CD1a+ LC. HHV-6 genome was detected in the biopsies of 5/19 patients with low copy number and viral Ag could not be detected in biopsies. CMV was not detected by PCR in this series.Conclusions/SignificanceTherefore, our findings do not support the hypothesis of a role of EBV, CMV, or HHV-6 in the pathogenesis of LCH, and indicate that the frequent detection of Epstein-barr virus (EBV) in Langerhans cell histiocytosis is accounted for by the infection of bystander B lymphocytes in LCH granuloma. The latter observation can be attributed to the immunosuppressive micro environment found in LCH granuloma.

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“…The clinical course tends to be indolent, but intra-abdominal tumors may have an aggressive progression [2,3]. This tumor is classified together with other histiocytic and reticulum cell tumors (Table 1) [4][5][6][7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Inflammatory pseudotumor-like follicular dendritic cell tumor: an underdiagnosed neoplasia

et al. 2017

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Follicular dendritic cell (FDC) tumor is an uncommon neoplasm. It generally presents as a slow-growing, painless mass, without systemic symptoms. Histological features usually include low grade spindle cell proliferation. This tumor occurs primarily in lymph nodes, especially cervical and axillary, however, involvement of extranodal sites such as the tonsils, spleen, liver, and gastrointestinal tract has been reported. Inflammatory pseudotumor-like follicular dendritic cell tumor (IPT-like FDCT) is a rare, distinctive histological subtype of this low-grade malignant neoplasm, with consistent Epstein-Barr virus (EBV) association. The differential diagnosis with other fibro-inflammatory tumor proliferations, as inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT), may be challenging. In the present article, two cases of IPT-like FDCT of the spleen are presented, with a broad overview of the literature: one 77-year-old male and one 70-year-old female. A large immunohistochemical panel should be used for diagnosis, as no single specific and totally sensitive markers are available, including markers for CD21, CD23, CD35, CNA42, and clusterin. Individual cases may express one or more of these markers, so that all of them should be investigated. In situ hybridization for EBV is constantly positive. Immunostaining for ALK should be negative, as it is present in roughly half of the cases of IMT. This panel should be used in combination of clinical, laboratory, and topographic evidences. Importantly, inclusion of this lesion as a possible option in clinical and pathological investigation represents the basis for a correct diagnosis.

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Langerhans cell histiocytosis and its relationship with Epstein-Barr virus

Cited by 8 publications

References 5 publications

Ethnicity, race, and socioeconomic status influence incidence of langerhans cell histiocytosis

Ethnicity, race, and socioeconomic status influence incidence of langerhans cell histiocytosis

Herpes-Virus Infection in Patients with Langerhans Cell Histiocytosis: A Case-Controlled Sero-Epidemiological Study, and In Situ Analysis

Inflammatory pseudotumor-like follicular dendritic cell tumor: an underdiagnosed neoplasia

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