Development of Langerhans cell histiocytosis associated with chronic active Epstein–Barr virus infection

Sakata, Naoki; Toguchi, Naomi; Kimura, Masatomo; Nakayama, Masahiro; Kawa, Keisei; Takemura, Tsukasa

doi:10.1002/pbc.21249

Cited by 23 publications

(31 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…EBV positive cells were always found in areas rich in B cells, and were labeled with either CD20, or CD79a antibodies (Figure 2), while no EBER+ cell labelled with CD1a, CD3, or CD68 cell was observed. These data indicate that EBV infects bystander B cells, which are present in a subsets of LCH granuloma [12]. Although positive controls were obtained, we did not observed immunoreactivity against HHV-6 in samples positive for HHV-6 DNA by PCR (data not shown), therefore the cellular target of HHV6 in LCH granuloma was not identified.…”

Section: Resultsmentioning

confidence: 75%

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

et al. 2008

View full text Add to dashboard Cite

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.

show abstract

Section: Resultsmentioning

confidence: 75%

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

et al. 2008

View full text Add to dashboard Cite

show abstract

“…Although factors such as infections (such as EBV, CMV, HHV-6), genetic disorders, malignancies (medulloblastoma, retinoblastoma, medullary astrocytoma, glioma), seasonal and environmental influences have been implicated in the pathogenesis of LCH 14, 15, 18, 19, 29, 30 , there is no evidence for one factor to play a pivotal role. 9, 11, 14, 31 However, each single factor may influence the immune system and thus predispose somewhat to LCH.…”

Section: Introductionmentioning

confidence: 99%

Langerhans Cell Histiocytosis of the Orbit: Five Clinicopathologic Cases and Review of the Literature

Herwig

Wojno

Zhang

et al. 2013

Survey of Ophthalmology

View full text Add to dashboard Cite

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. Herein, we describe the clinical and histologic spectrum of LCH of the orbit on the basis of five cases. One patient exhibited multifocal unisystem disease, the other four patients presented with a localized process. The typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunostaining. Transmission electron microscopy exhibited characteristic intracytoplasmic Birbeck granules. The different ophthalmic manifestations of LCH and treatment strategies are reviewed in the context of previously reported cases. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH has to be included in the differential diagnosis in tumors of the ocular adnexae, in particular in young children.

show abstract

“…Via cell-specific gene expression studies it has been proposed that pathological LC in LCH derive from bone marrow derived myeloid dendritic cell precursors and migrate to sites of LCH suggesting CD207 (langerin) is inducible from a bone-marrow source 3 . Other analyses of LCH being associated with viral infections, such as Epstein Barr virus, cytomegalovirus and other human herpes viruses have borne out mixed results 4–7 . Supporting a potential reactive etiology for LCH, numerous cases of benign Langerhans cell hyperplasia have been reported with scabies infestation 8–10 .…”

Section: Discussionmentioning

confidence: 99%

Langerhans Cell Hyperplasia From Molluscum Contagiosum

Hatter

Zhou

Honda

et al. 2015

The American Journal of Dermatopathology

View full text Add to dashboard Cite

Langerhans cell histiocytosis (LCH) carries a prognosis which ranges from benign to potentially fatal. There is currently little framework to decipher metrics which predict the benign versus aggressive nature of LCH. We wanted to determine if molluscum contagiosum virus (MCV) DNA could be isolated from a cutaneous lesion demonstrating Langerhans cell hyperplasia resembling LCH in a patient with both. We performed polymerase chain reaction (PCR) on biopsy proven MCV and the hyperplastic lesion. Two specific regions within the MCV genome were detected from both biopsies. We report our findings and suggest that some MCV can produce histologic lesions resembling LCH, similar to the literature on scabies mimicking LCH. Efforts to find a reactive “driver” in LCH may significantly inform the clinical scenario.

show abstract

Development of Langerhans cell histiocytosis associated with chronic active Epstein–Barr virus infection

Cited by 23 publications

References 20 publications

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

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

Langerhans Cell Histiocytosis of the Orbit: Five Clinicopathologic Cases and Review of the Literature

Langerhans Cell Hyperplasia From Molluscum Contagiosum

Contact Info

Product

Resources

About