Nodal cytotoxic molecule (CM)‐positive Epstein–Barr virus (EBV)‐associated peripheral T cell lymphoma (PTCL): a clinicopathological study of 26 cases

Abstract

Kato S, Takahashi E, Asano N, Tanaka T, Megahed N, Kinoshita T & Nakamura S (2012) Histopathology 61, 186–199 Nodal cytotoxic molecule (CM)‐positive Epstein–Barr virus (EBV)‐associated peripheral T cell lymphoma (PTCL): a clinicopathological study of 26 cases Aims: The clinicopathological distinctiveness of nodal cytotoxic molecule (CM)‐positive Epstein–Barr virus (EBV)‐associated peripheral T cell lymphoma (PTCL) remains to be clarified. We investigated 26 patients with this lymphoma compared to nodal CM+ EBV− PTCL (n = 39) and extranasal natural killer/T cell lymphoma of nasal type (ENKTL, n = 44). Methods and results: Nodal CM+ EBV+ PTCL patients were more likely to have B symptoms (P = 0.019) and hepatic involvement (P = 0.026) than nodal CM+ EBV− PTCL patients. The former also had more Stage III/IV disease (P = 0.025) but much less cutaneous involvement (P < 0.001) than ENKTL patients at diagnosis. This nodal EBV+ lymphoma possessed a distinctive immunophenotype of high CD8+, CD56− pattern with an aggressive clinical course (median, 6.6 months). Thrombocytopenia was present in 11 (50%) patients and found to be the strongest prognostic indicator (P = 0.001) in this nodal EBV+ group. For all nodal CM+ PTCL cases CD5 negativity, but not EBV positivity, was the significant adverse prognostic factor (P < 0.002) in a multivariate analysis, independent of prognostic index for PTCL (PIT) or International Prognostic Index (IPI) scores. Conclusions: Nodal CM+ EBV+ PTCL constitutes a unique group of lymphomas distinct from ENKTL. The data provide support for our assertion that nodal CM+ PTCL should be distinguished in the 2008 WHO category of PTCL, not otherwise specified.

Epstein-Barr virus–positive nodal T/NK-cell lymphoma: an analysis of 15 cases with distinct clinicopathological features

“…Thus, we preferred EBV-positive nodal T/NK-cell lymphoma to nodal involvement of extranodal lymphoma in these patients. In addition, because EBV-positive cells have been encountered in variable proportions in EBV-positive nodal T-cell lymphomas, we adopted a threshold of EBV positivity of at least 30% of neoplastic cells, in accordance with a recent study [8]. In fact, all cases in our study showed unequivocal EBV positivity ranging from 40% to 80% of tumor cells.…”

“…Although the existence of EBV-positive nodal T/NK-cell lymphoma has been recognized by many hematopathologists [3,8,9,[12][13][14], only a few case series are available on this disease; therefore, the clinicopathological characteristics of EBV-positive nodal T/NK-cell lymphoma remain to be clarified. This paucity might be attributable to the rarity of EBV-positive nodal T/NK-cell lymphoma and the lack of consensus criteria for the diagnosis of EBV-positive nodal T/NK-cell lymphoma.…”

“…In addition, several histologically unique lymphomas such as Lennert lymphoma and a follicular variant of PTCL are regarded as PTCL-NOS by the current World Health Organization (WHO) classification [6,7]. Recently, a few reports have demonstrated that Epstein-Barr virus (EBV)-positive nodal PTCL or EBV-positive nodal T-or NK (T/NK hereafter)-cell lymphoma is another unique entity of mature T/NK-cell lymphomas under the PTCL-NOS category [8,9].…”

Polymyositis as a Paraneoplastic Syndrome in Cytotoxic Molecule-positive and Epstein-Barr Virus-associated Peripheral T-cell Lymphoma, Not Otherwise Specified

“…However, in recent years, nodal cytotoxic molecule-positive and EBV-associated PTCL-NOS has been supposed to be distinct from the current category of mature T/natural killer (NK) cell lymphomas, because of its unique immunophenotypes and aggressive clinical course (9). The present case, therefore, would be categorized into this type of PTCL-NOS, due to its similar phenotype and clinical picture, although our case was an extra-nodal type.…”

Extranodal NK/T-cell lymphoma: a review of literature and case report

“…Приблизительно в четверти слу-чаев ЭНКТЛ может наблюдаться поражение костного мозга и лейкемизация опухолевого процесса [15]. Пер-вичное поражение лимфатических узлов (ЛУ) без экс-транодальных поражений при ЭНКТЛ встречается крайне редко, в таких случаях необходимо проводить дифференциальную диагностику с цитотоксической нодальной Т-клеточной лимфомой, ассоциированной с вирусом Эпштейна-Барр (ВЭБ) [16].…”