Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma

Massone

The American Journal of Dermatopathology

et al. 2016

Hydroa vacciniforme-like lymphoma is a recently recognized cutaneous T-cell lymphoma associated with Epstein-Barr virus. The disease is observed in children of Latin American or Asian ethnicity. The authors report the clinical, histopathological, and immunophenotypical features of 9 new Mexican patients (M:F = 2:1; mean age, 14.5 years; median age, 13.3 years; age range, 4-27 years), expanding on previous observations of this elusive disease. The most common clinical aspects were persistent facial edema with necroses and pitted scars. Histopathological analyses revealed variably dense lymphoid infiltrates with common angiodestructive features. Neoplastic cells expressed CD3 and cytotoxic markers in all cases and were constantly positive for Epstein-Barr virus (EBER-1). Expression of other markers was variable. Follow-up data revealed that all patients died within 6 months or less, thus showing a very aggressive course with poor prognosis.

Section: Discussionsupporting

confidence: 86%

“…2 Nowadays, it is clear that it represents a primary CTCL with a prolonged but usually fatal course. 6,7,17 In this article, we studied the clinicopathologic and phenotypic features of 9 additional patients with HVLL, reporting interesting features that expand on the knowledge about this rare and peculiar disease.…”

Section: Introductionmentioning

confidence: 97%

Clinicopathologic Features of Hydroa Vacciniforme–Like Lymphoma

Massone

The American Journal of Dermatopathology

et al. 2016

“…8,38 These observations are not unexpected, since other EBV-associated neoplasms such as nasopharyngeal carcinoma and NK/T-cell nasal lymphomas have been shown to be more prevalent in Asian and Latin American populations. 39,40 Beltran et al recently described a group of patients with EBV1 DLBCL, who displayed the histological and immunophenotypical characteristics described for this entity, but the 50 years cutoff criteria was not fulfilled. 6 Moreover, Oyama et al excluded seven patients younger than 40 years of age (7% of their EBV1 DLBCL patients), 8 whereas a report from Korea by Park et al included patients with EBV1 DLBCL as young as 20 years.…”

Section: Discussionmentioning

confidence: 99%

Epstein-Barr virus-positive diffuse large B-cell lymphoma association is not only restricted to elderly patients

Cohen

Narbaitz²,

Metrebian³

et al. 2014

Int. J. Cancer

Diffuse large B-cell lymphoma (DLBCL), the most common group of malignant lymphomas, account for 30% of adult nonHodgkin lymphomas. The 2008 World Health Organization (WHO) classification included a new entity, Epstein-Barr virus (EBV)1 DLBCL of the elderly, affecting patients aged 50 years or older. However, some reports of younger EBV1 DLBCL cases, without evidence of underlying immunosuppression, can be found. The role of EBV in tumor microenvironment composition in DLBCL is still not well understood. Our aim was to assess EBV presence and latency pattern as well as tumor T-cell population in an adult DLBCL series of Argentina. The study was conducted on biopsies from 75 DLBCL patients. EBERs expression was performed by in situ hybridization, while EBV gene expression was analyzed using real-time polymerase chain reaction. LMP1, LMP2A, EBNA2, EBNA3A, CD4, CD8 and Foxp3 expression was assessed by immunohistochemistry. Nine percent of cases showed EBV expression, with similar frequency among patients younger than 50 years and 50 years or older (13% and 8%, respectively). T-cell subsets were not altered by EBV presence. Latency type II was the most frequently observed, together with lytic gene expression in EBV1 DLBCL, with 20% of EBERs1 cells. These findings suggest that EBV1 DLBCL in our series was similar to the previously described in Asia and Latin-America, displaying latency II or III expression profile and no agespecific characteristics. Finally, EBV1 DLBCL may be an entity that is not only restricted to patients who are older than 50 years of age, in consequence the age cutoff revision may be a current goal.

“…Chronic active EBV infection of T/NK type shows a broad range of clinical manifestations from indolent, localized forms like hydroa vacciniforme-like LPD and severe mosquito bite allergy to a more systemic form characterized by fever, hepatosplenomegaly, and lymphadenopathy with or without cutaneous manifestations. 140,141 Systemic EBV 1 TCL of childhood, no longer referred to as a "lymphoproliferative disorder," has a fulminant clinical course usually associated with a hemophagocytic syndrome. The differential diagnosis includes acute EBV-associated hemophagocytic lymphohistiocytosis (HLH), which can present acutely, but in some patients responds well to the HLH 94 protocol, and is not considered neoplastic.…”

Section: Ebv 1 T-cell and Nk-cell Lymphomasmentioning

confidence: 99%

The 2016 revision of the World Health Organization classification of lymphoid neoplasms

Swerdlow

Campo

Pileri

et al. 2016

Blood

6,860

6,518

A revision of the nearly 8-year-old World Health Organization classification of the lymphoid neoplasms and the accompanying monograph is being published. It reflects a consensus among hematopathologists, geneticists, and clinicians regarding both updates to current entities as well as the addition of a limited number of new provisional entities. The revision clarifies the diagnosis and management of lesions at the very early stages of lymphomagenesis, refines the diagnostic criteria for some entities, details the expanding genetic/molecular landscape of numerous lymphoid neoplasms and their clinical correlates, and refers to investigations leading to more targeted therapeutic strategies. The major changes are reviewed with an emphasis on the most important advances in our understanding that impact our diagnostic approach, clinical expectations, and therapeutic strategies for the lymphoid neoplasms.