Clinical and Histopathological Features of an Italian Monocentric Series of Primary Small Bowel T-Cell Lymphomas

Abstract

The gastrointestinal (GI) tract is the most common extranodal site of occurrence of non-Hodgkin lymphomas. Most GI lymphomas are of B-cell lineage, while T-cell lymphomas are less frequent. The aim of our retrospective study was to depict the clinical-pathological profile of a series of patients affected by intestinal T-cell lymphomas (ITCL) and possibly define hallmarks of these neoplasms. A total of 28 patients were included: 17 enteropathy-associated T-cell lymphomas (EATL), 5 monomorphic epitheliotropic T-cell lymphomas (MEITL), 3 indolent T-cell lymphoproliferative disorders of the gastrointestinal tract (ITCLDGT), and 3 intestinal T-cell lymphomas not otherwise specified (ITCL-NOS). Celiac disease (CD) was diagnosed in around 70% of cases. Diagnosis of EATL showed a significant correlation with CD30 expression, whereas MEITL with angiotropism and CD56 positivity. ITCLDGT cases showed plasma cells infiltration. Peripheral lymphocytosis, the absence of a previous diagnosis of CD, an advanced Lugano clinical stage, and the histological subtype ITCL-NOS were significantly associated with worse survival at multivariate analysis. Our findings about the epidemiological, clinical, and histopathological features of ITCL were in line with the current knowledge. Reliable prognostic tools for these neoplasms are still lacking but according to our results lymphocytosis, diagnosis of CD, Lugano clinical stage, and histological subtype should be considered for patient stratification.

Keywords: EATL; ITCL; ITCLDGT; MEITL; celiac disease; lymphoproliferative disorders; small bowel lymphoma.

Figure 1

Kaplan–Meier curves showing difference in survival in patients with intestinal T-cell lymphomas (ITCL) involving the small bowel, according to clinical variables. Differences were compared with the log-rank test, and a two-sided p-value < 0.05 was considered statistically significant. (A) Patients with low lymphocyte counts show better survival than those with marked lymphocytosis (lymphocytes > 4 × 10³/μL); (B) Patients with no history of celiac disease (CD) show worse survival than those with previous CD diagnosis.

Figure 2

Kaplan–Meier curves showing difference in survival in patients with intestinal T-cell lymphomas (ITCL) involving the small bowel, according to histopathological subtypes. Differences between the four curves were evaluated with the log-rank test and a two-sided p-value < 0.01 was found, showing that the variant with the worst prognosis was intestinal T-cell lymphoma not otherwise specified (ITCL-NOS), while the one with the best prognosis was the indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (ITCLDGT).

Figure 3

Enteropathy-associated T-cell lymphoma (EATL). Hematoxylin–eosin staining ((A), 20×) shows a diffuse polymorphic ((B), 400×) proliferation of T-cells frequently expressing CD3 ((C), 20×) and, in most of our cases, CD30 ((D), 200×).

Figure 4

Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (ITCLDGT). Hematoxylin–eosin staining ((A), 20×) shows a proliferation of small to medium-sized ((B), 400×) T-cells CD3+ ((C), 100×) and with a low proliferative index ((D), 100×). In 2/3 cases, the background infiltrate was rich in CD138+ plasma cells ((E), 100×).