EBV-associated Cutaneous NK/T-cell Lymphoma

Abstract

We have reviewed clinically, morphologically, and immunophenotypically a series of 14 Epstein-Bar virus (EBV)+ cutaneous natural killer cell (NK)/T-cell lymphoma from Peru. Most (11 out of 14) of these cases fit well into the category of Hydroa vacciniforme-like lymphoma (HVLL), but 3 have a different clinical presentation, without facial involvement. In all 14 cases, skin lesions present in both the sun-exposed and nonexposed areas exhibited a slowly progressive relapsing course, changing from edema, to blistering, ulceration, and final scarring. The immunophenotype had a cytotoxic T or NK-cell lineage. The mean time of disease before admission to hospital was 69 months (range, 6 mo to 31 y). Only 2 patients had fever, hepatosplenomegaly, systemic lymphadenopathy, and a high lactate dehydrodenage (LDH) level at the time of diagnosis, whereas 10 had facial swelling. After treatment, only 4 patients remain alive, although with persistent disease. Ten patients died after a mean follow-up of 11.6 months after the initial diagnosis (range, 1 to 32 mo), because of concurrent infections (4 cases), disease progression (4 patients) or both (2 patients). Endemic Epstein-Bar virus (EBV)-positive cutaneous NK/T-cell lymphoproliferative disorders in childhood and early adulthood are characterized by a protracted clinical course, eventually leading to an aggressive phase characterized by concurrent infections and disease progression.

Oral and cutaneous lymphomas other than mycosis fungoides and sézary syndrome in a mexican cohort: Recategorization and evaluation of international geographical disparities

“…The former was led by CD30+ lymphoprolifeative disorders, in accordance with other international reports,[2829303132333435363738394041] followed closely by EBV-related NK/T-cell malignancies (PC-NK/TCL and HVLLPD), commonly reported in Asia and Central America but exceptionally in Europe and the USA. [3132333435363738394041434445464748] This comparatively higher frequency could potentially be explained by previously described higher prevalence of EBV subtypes associated to human pathology in developing countries and plausibly to the onset of infection at an earlier age. [49] The same argument could hold true, to the existence of one case of EBV+ DLBCL NOS among our small cohort of 7 PC-BCL, reportedly one of the rarest entities among all PCL.…”

“…[30] While only one-third of the non-MF/SS PCL in our laboratory could be properly reclassified due to limited tissue availability, the frequency of lymphoma variants seems to differ in some aspects from both European/USA and South American registries. [282930313233343536373839404142] As in other international cohorts, non-MF/SS PC-TCL outnumbered PC-BCL. The former was led by CD30+ lymphoprolifeative disorders, in accordance with other international reports,[2829303132333435363738394041] followed closely by EBV-related NK/T-cell malignancies (PC-NK/TCL and HVLLPD), commonly reported in Asia and Central America but exceptionally in Europe and the USA.…”

“…Surprisingly, no cases of PC-MZL were observed despite being reported as the second most common form of PC-BCL in European, USA, and South American studies, and speculated to be closely linked in its pathogenesis to microbial and/or autoimmune antigenic triggers, with potential geographical restrictions. [8282930313233343536373839404142434445464748] This apparent null incidence in our cohort could be artificial; however, since PC-MZL was recently described, and formerly reported in our institution as B-cell predominant cutaneous pseudolymphoma (cutaneous lymphoid hyperplasia) before ISH for immunoglobulin light chains became available. Similarly, our cohort lacks cases of PC CD4+ small/medium T-cell lymphoproliferative disorder which until recently would have likely been diagnosed in our institution as pseudolymphomatous folliculitis but is thought to represent one of the most common non-MF/SS PCL in some recent series.…”

“…[282930313233343536373839404142] As in other international cohorts, non-MF/SS PC-TCL outnumbered PC-BCL. The former was led by CD30+ lymphoprolifeative disorders, in accordance with other international reports,[2829303132333435363738394041] followed closely by EBV-related NK/T-cell malignancies (PC-NK/TCL and HVLLPD), commonly reported in Asia and Central America but exceptionally in Europe and the USA. [3132333435363738394041434445464748] This comparatively higher frequency could potentially be explained by previously described higher prevalence of EBV subtypes associated to human pathology in developing countries and plausibly to the onset of infection at an earlier age.…”

“…To date, only a handful of studies on the descriptive epidemiology of PCL have been conducted in Latin America (mainly Brazil, Peru, and Argentina),[31323334353637383940] and not, to the best of our knowledge, in Central and Northern Latin America where ethnicity and incidence of EBV and human T-cell leukemia virus I/II (HTLV-I/II) seropositivity substantially differ.…”