Clinicopathologic features of NK-cell enteropathy
Case . | Age, y . | Sex . | Symptoms . | GI involvement . | Endoscopic . | Tumor content, % . | CD56 . | CD2 . | CD3 . | CD7 . | Ki-67 . | pSTAT5 . | TCRG PCR . | Follow-up, months . | Status . |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 65 | M | Reflux | S | Diffuse erythema and a polyp in antrum and body of stomach | 50 | P | N | Cyto | P | 75-90% | ND | Polyclonal | 45 | Alive, no rebiopsy, treated for H pylori |
2 | 76 | F | Diverticulosis, stool blood test + | S, D | Multiple gastric ulcers and duodenal ulcers, erosive gastritis | 20 | P | P | Cyto | P | 25-50% | P | Polyclonal | 48 | Alive, rebiopsies showed persistent disease, no treatment |
3 | 31 | M | None; family history of colon cancer | C, S | Not known | 20 | P | N | Cyto | N | 25-50% | P | Polyclonal | 84 | Alive with rebiopsies showed persistent disease, treatment unclear |
4 | 63 | F | Upper GI discomfort, mild duodenal wall thickening on abdominal computed tomography | D | Duodenal polyp with erosion | 30 | P | N | Cyto | ND | 25-50% | P | ND | Lost follow-up | |
5 | 71 | F | Not known | S, C | Ulceration in colon and terminal ileum | 20 | P | P | Cyto | P | 25-50% | ND | Restricted | 96 | Rebiopsies showed persistent disease for 4 y, but last 1 unremarkable, treatment unclear |
6 | 48 | F | 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 | P | Polyclonal | 95 | Alive, no rebiopsy, no treatment |
7 | 69 | F | Recurrent gastric and duodenal ulcers, prior history of H pylori | S, D | Not known | 50 | P | P | Cyto | P | 50-75% | P | Polyclonal | Lost follow-up | |
8 | 9 | F | 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 | P | partial | Partial; cyto | ND | ND | P | ND | 47 | Subcutaneous methotrexate, healthy |
9 | 38 | M | History of PSC, colonoscopy done for PSC work up to rule out IBD | C, TI | Ulcers and papules from ileum to the rectum | 30 | P | 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 | F | Upper GI discomfort, PET SUV 4 of gastric fundus; outside called NK/T-cell lymphoma, consult for HSCT and chemotherapy | S | Ulcer | 40 | P | P | Cyto | P | 25-50% | P | Polyclonal | 24 | Alive, no rebiopsy or treatment |
Case . | Age, y . | Sex . | Symptoms . | GI involvement . | Endoscopic . | Tumor content, % . | CD56 . | CD2 . | CD3 . | CD7 . | Ki-67 . | pSTAT5 . | TCRG PCR . | Follow-up, months . | Status . |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 65 | M | Reflux | S | Diffuse erythema and a polyp in antrum and body of stomach | 50 | P | N | Cyto | P | 75-90% | ND | Polyclonal | 45 | Alive, no rebiopsy, treated for H pylori |
2 | 76 | F | Diverticulosis, stool blood test + | S, D | Multiple gastric ulcers and duodenal ulcers, erosive gastritis | 20 | P | P | Cyto | P | 25-50% | P | Polyclonal | 48 | Alive, rebiopsies showed persistent disease, no treatment |
3 | 31 | M | None; family history of colon cancer | C, S | Not known | 20 | P | N | Cyto | N | 25-50% | P | Polyclonal | 84 | Alive with rebiopsies showed persistent disease, treatment unclear |
4 | 63 | F | Upper GI discomfort, mild duodenal wall thickening on abdominal computed tomography | D | Duodenal polyp with erosion | 30 | P | N | Cyto | ND | 25-50% | P | ND | Lost follow-up | |
5 | 71 | F | Not known | S, C | Ulceration in colon and terminal ileum | 20 | P | P | Cyto | P | 25-50% | ND | Restricted | 96 | Rebiopsies showed persistent disease for 4 y, but last 1 unremarkable, treatment unclear |
6 | 48 | F | 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 | P | Polyclonal | 95 | Alive, no rebiopsy, no treatment |
7 | 69 | F | Recurrent gastric and duodenal ulcers, prior history of H pylori | S, D | Not known | 50 | P | P | Cyto | P | 50-75% | P | Polyclonal | Lost follow-up | |
8 | 9 | F | 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 | P | partial | Partial; cyto | ND | ND | P | ND | 47 | Subcutaneous methotrexate, healthy |
9 | 38 | M | History of PSC, colonoscopy done for PSC work up to rule out IBD | C, TI | Ulcers and papules from ileum to the rectum | 30 | P | 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 | F | Upper GI discomfort, PET SUV 4 of gastric fundus; outside called NK/T-cell lymphoma, consult for HSCT and chemotherapy | S | Ulcer | 40 | P | P | Cyto | P | 25-50% | P | 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.