Table 1.

Clinicopathologic features of NK-cell enteropathy

CaseAge, ySexSymptomsGI involvementEndoscopicTumor content, %CD56CD2CD3CD7Ki-67pSTAT5TCRG PCRFollow-up, monthsStatus
65 Reflux Diffuse erythema and a polyp in antrum and body of stomach 50 Cyto 75-90% ND Polyclonal 45 Alive, no rebiopsy, treated for H pylori 
76 Diverticulosis, stool blood test + S, D Multiple gastric ulcers and duodenal ulcers, erosive gastritis 20 Cyto 25-50% Polyclonal 48 Alive, rebiopsies showed persistent disease, no treatment 
31 None; family history of colon cancer C, S Not known 20 Cyto 25-50% Polyclonal 84 Alive with rebiopsies showed persistent disease, treatment unclear 
63 Upper GI discomfort, mild duodenal wall thickening on abdominal computed tomography Duodenal polyp with erosion 30 Cyto ND 25-50% ND Lost follow-up  
71 Not known S, C Ulceration in colon and terminal ileum 20 Cyto 25-50% ND Restricted 96 Rebiopsies showed persistent disease for 4 y, but last 1 unremarkable, treatment unclear 
48 Persistent diarrhea, outside called T-cell lymphoma on small bowel resection. Received 1 cycle of chemotherapy S, D, and TI Not known 15 Indeterminate ND Cyto ND ND Polyclonal 95 Alive, no rebiopsy, no treatment 
69 Recurrent gastric and duodenal ulcers, prior history of H pylori S, D Not known 50 Cyto 50-75% Polyclonal Lost follow-up  
Abdominal pain, daily vomit, failure to gain weight since age 6 y (for past 3 y) S, proximal small intestine Hyperemia and multiple ulcers in the stomach and multiple ulcers and stenosis in the duodenum 60 partial Partial; cyto ND ND ND 47 Subcutaneous methotrexate, healthy 
38 History of PSC, colonoscopy done for PSC work up to rule out IBD C, TI Ulcers and papules from ileum to the rectum 30 ND Cyto ND 75-90% suboptimal Restricted 71 Liver transplant for PSC 4 y later. Rebiopsy before the transplant showed persistent disease. On immunosuppression but asymptomatic and PET was negative 
10 59 Upper GI discomfort, PET SUV 4 of gastric fundus; outside called NK/T-cell lymphoma, consult for HSCT and chemotherapy Ulcer 40 Cyto 25-50% Polyclonal 24 Alive, no rebiopsy or treatment 
CaseAge, ySexSymptomsGI involvementEndoscopicTumor content, %CD56CD2CD3CD7Ki-67pSTAT5TCRG PCRFollow-up, monthsStatus
65 Reflux Diffuse erythema and a polyp in antrum and body of stomach 50 Cyto 75-90% ND Polyclonal 45 Alive, no rebiopsy, treated for H pylori 
76 Diverticulosis, stool blood test + S, D Multiple gastric ulcers and duodenal ulcers, erosive gastritis 20 Cyto 25-50% Polyclonal 48 Alive, rebiopsies showed persistent disease, no treatment 
31 None; family history of colon cancer C, S Not known 20 Cyto 25-50% Polyclonal 84 Alive with rebiopsies showed persistent disease, treatment unclear 
63 Upper GI discomfort, mild duodenal wall thickening on abdominal computed tomography Duodenal polyp with erosion 30 Cyto ND 25-50% ND Lost follow-up  
71 Not known S, C Ulceration in colon and terminal ileum 20 Cyto 25-50% ND Restricted 96 Rebiopsies showed persistent disease for 4 y, but last 1 unremarkable, treatment unclear 
48 Persistent diarrhea, outside called T-cell lymphoma on small bowel resection. Received 1 cycle of chemotherapy S, D, and TI Not known 15 Indeterminate ND Cyto ND ND Polyclonal 95 Alive, no rebiopsy, no treatment 
69 Recurrent gastric and duodenal ulcers, prior history of H pylori S, D Not known 50 Cyto 50-75% Polyclonal Lost follow-up  
Abdominal pain, daily vomit, failure to gain weight since age 6 y (for past 3 y) S, proximal small intestine Hyperemia and multiple ulcers in the stomach and multiple ulcers and stenosis in the duodenum 60 partial Partial; cyto ND ND ND 47 Subcutaneous methotrexate, healthy 
38 History of PSC, colonoscopy done for PSC work up to rule out IBD C, TI Ulcers and papules from ileum to the rectum 30 ND Cyto ND 75-90% suboptimal Restricted 71 Liver transplant for PSC 4 y later. Rebiopsy before the transplant showed persistent disease. On immunosuppression but asymptomatic and PET was negative 
10 59 Upper GI discomfort, PET SUV 4 of gastric fundus; outside called NK/T-cell lymphoma, consult for HSCT and chemotherapy Ulcer 40 Cyto 25-50% Polyclonal 24 Alive, no rebiopsy or treatment 

C, colon; D, duodenum; F, female; M, male; N, negative; ND, not done; P, positive; PSC, primary sclerosing cholangitis; S, stomach; SUV, standardized uptake value; TI, terminal ileum.

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