Contributions
Abstract: EP531
Type: E-Poster Presentation
Session title: Aggressive Non-Hodgkin lymphoma - Clinical
Background
MEITL is a rapidly progressive rare extranodal T-cell lymphoma that arises from the intestinal intraepithelial T lymphocytes. In its 2016 classification, the WHO established MEITL as a separate entity of what was previously known as type 2 enteropathy-associated T-cell lymphoma. While morphologic characteristics are codified in its name, this entity tends to have its own clinicopathologic characteristics.
Aims
We conducted this analysis to explore the clinicopathologic descriptors and prognosticators of this little-known newly established T-cell entity.
Methods
In order to study the demographic characteristics, molecular and immunohistochemical signatures, therapeutic interventions, survival, and prognostic factors, we compiled a pooled database of 116 cases. Descriptive statistics were calculated. Cox proportional-hazards model and Log-rank tests were used to assess the influence of demographic and clinicopathologic factors on overall survival (OS).
Results
A total of 116 patients with confirmed MEITL were identified. The median age was 59.5 years with a peak incidence between ages 56 and 68. There was a male predominance with M:F ratio of 2. As the only involved gastrointestinal (GI) site, Jejunum was the most involved site of the disease (Jejunum (J) 21%, Duodenum (D) 7%, Colorectal (CRC) 10%, Ileum (I) 6%, Gastric (G) 2%). Jejunum was also the most involved site when the disease involved multiple GI sites (J 71%, I 57%, D 46%, C 30%, R 3%). The most frequently involved anatomic combinations were multifocal small intestine 26%, small intestine with colorectal 21%, J&I 7%, D&J 2%. While 25% of patients presented with lymphadenopathy (79% regional, 14% distal, 7% both), only 6% had bone marrow involvement and none had hepatosplenomegaly. Extra-nodal and extra-gastrointestinal involvement comprised lung (5%), CNS (3%), and skin (1%). Stage distribution consisted of Stage I (69%), II (9%), III (6%), IV (16%). Most cases expressed CD2 (77%), CD3 (100%), CD7 (86%), CD8 (80%), CD56 (89%), CD103 (86%), and Granzyme-B (52%). MEITL cases expressed TCR-αβ (39%), γδ (56%), and null (5%). There were no significant differences in age, sex, and CD profiles with respect to TCR-status. The most common clinical presentations were abdominal pain, followed by perforation, diarrhea, and weight loss. The median duration of symptoms prior to diagnosis was 4 months. Median OS of the whole group was 11 months. Compared to no treatment, combination chemotherapy and stem cell transplant (SCT) were statistically superior with a median OS of 2, 9, and 34 months respectively (p=0.0005). Furthermore, quality of response and surgical resection imparted a survival advantage. OS was not impacted by sex, presentation with obstruction or perforation, or anatomic site involvement. While older age, weight loss, and TCRγδ seemed to negatively impact OS, they did not reach statistical significance.
Conclusion
This study presents a comprehensive clinicopathologic data from a pooled cohort of patients with MEITL. It describes the clinicopathologic features of MEITL and identifies major determinants of OS in this rare disease.
Keyword(s): Clinical data, Prognostic factor, T cell lymphoma
Abstract: EP531
Type: E-Poster Presentation
Session title: Aggressive Non-Hodgkin lymphoma - Clinical
Background
MEITL is a rapidly progressive rare extranodal T-cell lymphoma that arises from the intestinal intraepithelial T lymphocytes. In its 2016 classification, the WHO established MEITL as a separate entity of what was previously known as type 2 enteropathy-associated T-cell lymphoma. While morphologic characteristics are codified in its name, this entity tends to have its own clinicopathologic characteristics.
Aims
We conducted this analysis to explore the clinicopathologic descriptors and prognosticators of this little-known newly established T-cell entity.
Methods
In order to study the demographic characteristics, molecular and immunohistochemical signatures, therapeutic interventions, survival, and prognostic factors, we compiled a pooled database of 116 cases. Descriptive statistics were calculated. Cox proportional-hazards model and Log-rank tests were used to assess the influence of demographic and clinicopathologic factors on overall survival (OS).
Results
A total of 116 patients with confirmed MEITL were identified. The median age was 59.5 years with a peak incidence between ages 56 and 68. There was a male predominance with M:F ratio of 2. As the only involved gastrointestinal (GI) site, Jejunum was the most involved site of the disease (Jejunum (J) 21%, Duodenum (D) 7%, Colorectal (CRC) 10%, Ileum (I) 6%, Gastric (G) 2%). Jejunum was also the most involved site when the disease involved multiple GI sites (J 71%, I 57%, D 46%, C 30%, R 3%). The most frequently involved anatomic combinations were multifocal small intestine 26%, small intestine with colorectal 21%, J&I 7%, D&J 2%. While 25% of patients presented with lymphadenopathy (79% regional, 14% distal, 7% both), only 6% had bone marrow involvement and none had hepatosplenomegaly. Extra-nodal and extra-gastrointestinal involvement comprised lung (5%), CNS (3%), and skin (1%). Stage distribution consisted of Stage I (69%), II (9%), III (6%), IV (16%). Most cases expressed CD2 (77%), CD3 (100%), CD7 (86%), CD8 (80%), CD56 (89%), CD103 (86%), and Granzyme-B (52%). MEITL cases expressed TCR-αβ (39%), γδ (56%), and null (5%). There were no significant differences in age, sex, and CD profiles with respect to TCR-status. The most common clinical presentations were abdominal pain, followed by perforation, diarrhea, and weight loss. The median duration of symptoms prior to diagnosis was 4 months. Median OS of the whole group was 11 months. Compared to no treatment, combination chemotherapy and stem cell transplant (SCT) were statistically superior with a median OS of 2, 9, and 34 months respectively (p=0.0005). Furthermore, quality of response and surgical resection imparted a survival advantage. OS was not impacted by sex, presentation with obstruction or perforation, or anatomic site involvement. While older age, weight loss, and TCRγδ seemed to negatively impact OS, they did not reach statistical significance.
Conclusion
This study presents a comprehensive clinicopathologic data from a pooled cohort of patients with MEITL. It describes the clinicopathologic features of MEITL and identifies major determinants of OS in this rare disease.
Keyword(s): Clinical data, Prognostic factor, T cell lymphoma