T-cell Receptor (TCR) Phenotype of Nodal Epstein-Barr Virus (EBV)-positive Cytotoxic T-cell Lymphoma (CTL)

Abstract

Among Epstein-Barr virus (EBV)-positive cytotoxic T/NK-cell lymphoma, there are only a few reports on the clinicopathologic features of patients with primary nodal presentation (nodal EBV cytotoxic T-cell lymphoma [CTL]). Here, we compared the clinicopathologic profiles of 39 patients with nodal EBV CTL with those of 27 cases of "extranasal" NK/T-cell lymphoma of nasal type (ENKTL), especially addressing their T-cell receptor (TCR) phenotype. Histologically, 22 of 39 nodal EBV CTL cases (56%) were unique in having centroblastoid appearance, which was contrasted with the lower incidence of this feature in ENKTL (15%, P=0.001). In contrast, pleomorphic appearance was more frequently seen in ENKTL than in nodal EBV CTL (67% vs. 23%, P=0.001). Thirty-three of 39 nodal EBV CTL cases (85%) were of T-cell lineage on the basis of TCR expression and/or TCRγ gene rearrangement; in detail, 18 cases (46%) were TCRβ positive (αβ T), 5 (13%) were TCRγ and/or δ positive (γδ T), and 10 (26%) were TCR-silent type with clonal TCRγ gene rearrangement but no expression of TCRβ, γ, or δ. These results were clearly contrasted by a lower incidence of T-cell lineage in ENKTL (7 cases, 26%, P<0.001). Notably, the survival time of the 5 nodal lymphoma patients with γδ T-cell phenotype was within 3 months, which was inferior to those of αβ T and TCR-silent types (P=0.003), and 3 of those with available clinical information were all found to be associated with autoimmune diseases. These data suggest that nodal EBV CTL is distinct from ENKTL.

Reappraisal of nodal Epstein‐Barr Virus‐negative cytotoxic T‐cell lymphoma: Identification of indolent CD5+ diseases

“…We identified 58 evaluable cases of nodal EBV‐negative CTL with paraffin blocks available for analyses, including 39 in our previous study . As a control group, we analyzed data from 48 nodal EBV‐positive CTL cases, including 39 previously reported …”

“…1,2,[18][19][20][21][22] We recently documented that nodal EBV-positive CTL has poor prognosis often with a cd phenotype. 7,8,23 On the other hand, recent studies shed a light on an indolent cytotoxic T-cell lymphoma/LPD, characterized by a TCRab phenotype and younger onset age, referred to as subcutaneous panniculitis-like T-cell lymphoma with an ab T-cell phenotype. [24][25][26][27] The available data suggest that CMs, EBV infection, and TCRab or cd phenotype status likely affect the pathophysiology of PTCL-NOS.…”

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

“…They most often present in older adults, and also can be seen in the posttransplant setting and other immunodeficiency states. 101,142,143 Hodgkin lymphomas…”

Case of primary central nervous system histiocytic sarcoma with prominent proliferation of histiocytic cells between the trabeculae of reactive glial cells

“…DNA was extracted from formalin‐fixed paraffin‐embedded sections. Rearrangements in the genes for immunoglobulin heavy chain (IGH) and T cell receptor (TCR) gamma (TCRγ) ( IGH and TCRγ , respectively) were identified by polymerase chain reaction (PCR) using the BIOMED2 protocol, as previously described . Direct sequencing was performed to identify BRAF mutations in V600E and phosphatidylinositide‐3 kibnase activating subunit (PIKC3A) mutations in E542K, E545K/D, and H1047R.…”