Gastrointestinal T Cell Lymphoma: Predominant Cytotoxic Phenotypes, Including Alpha/Beta, Gamma/Delta T Cell and Natural Killer Cells

Abstract

Gastrointestinal T cell lymphoma (TCL) is a rare subset of peripheral TCL, presenting with or without cytotoxic phenotype, a history of coeliac disease (CD) and enteropathy. However, CD is rare in Japan. Here, we describe the clinicopathological features of 18 Japanese cases. Lesions were found in the small intestine (n=13), stomach (n=3) and colon (n=2). Seven patients presented with enteropathy but none had a history of CD. Lymphomas appeared as ulceration (n=11), tumour formation (n=6), or polypoid growth (n=1). Histologically (REAL classification), neoplastic lesions were composed of intestinal type T cell lymphoma (ITCL, n=13, including one case with NK type), anaplastic large cell (ALCL, n=2), adult T cell leukaemia/lymphoma (ATLL, n=2), and lymphoblastic type (n=1). Epstein Barr virus infection was detected by EBER-1 in situ hybridization in 6 of 11 cases with ITCL but not in the other types. ALCL expressed CD30. CD56 was expressed in 3 of 11 cases of ITCL but not in other types. Among the 10 examined cases, 8 were alphabeta T cell type [CD2+, CD3+, T cell receptor (TCR)delta-1-, betaF1+], one was gammadelta T cell type [CD2+, CD3+, TCRdelta-1+, betaF1-], and the remaining case expressed natural killer (NK) cell type [CD2+, CD3-, CD56+, TCRdelta-1-, betaF1-]. Among the 8 examined cases, 3 expressed CD103 molecule, which was associated with extrathymic T cells of intraepithelial lymphocytes. All cases except ATLL expressed the cytotoxicity-associated molecule of TIA-1, and 11 of 14 TIA-1 positive cases expressed activated cytotoxic molecules of perforin, granzyme B, and/or Fas ligand. Despite the morphological, genetic and phenotypic heterogeneity, prognosis was poor, and 11 of 13 patients with small intestinal lesions died albeit appropriate treatment, but 3 of 4 patients with gastric or colonic lesions were still alive. The main cause of death was intestinal perforation. The latter might be due to the site specificity of small intestine and tumour cytotoxicity.

Long-term Remission of Primary Gastric T Cell Lymphoma Associated with Human T Lymphotropic Virus Type 1: A Report of Two Cases and Review of the Literature

“…To date, 22 cases of primary gastric T-cell lymphoma associated with HTLV-1 have been reported in the English literature (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). Table 1 summarizes the clinicopathologic features of these 22 cases and the present 2 cases.…”

“…While systemic adult T-cell leukemia or lymphoma (ATLL), which are caused by human T lymphotropic virus type 1 (HTLV-1), often involves the gastrointestinal tract (3,4), primary gastric T-cell lymphoma associated with HTLV-1 is extremely rare (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). In addition, primary gastric T-cell lymphomas, especially those associated with HTLV-1, generally show an extremely poor prognosis (19).…”

CD56 Positive Intestinal T-Cell Lymphoma: Treatment with High Dose Chemotherapy and Autologous Peripheral Blood Stem Cell Transplantation.

“…Chott et al reported that 21% ofITCL were CD56+(3). Three of eleven Japanese cases of ITCL were reported to be CD56positive (10). Some overlapping histological and clinical features between CD56+and CD56-cases indicate that the former and the latter belong to the same clinicopathological entity of ITCL.…”

Gastrointestinal lymphoma

“…The 5-year failure-free survival and overall survival rates were 3.2% and 19.7%, respectively. Katoh et al [59] described 18 cases of GI T-cell lymphomas from Japan. Thirteen patients had enteropathy-type T-cell lymphomas and two had adult T-cell lymphoma associated with human T-cell leukemia virus type I infection [1].…”