P062  Lymphoma masquerading as Crohn’s Disease in an 8 year old pediatric patient

P062  Lymphoma masquerading as Crohn’s Disease in an 8 year old pediatric patient

 

Beri Jonathan1, Corao Diana1, Powell Jonathan1, Delrosario Fernando1, Molle-Rios Zarela1
1 Nemours - Alfred I duPont Hospital for Children, Wilmington, United States

CASE: We present an 8 year old boy with history of periumbilical abdominal pain, intermittent diarrhea, and a four month, 9 lb weight loss. An EGD and colonoscopy with ileum intubation appeared normal with histology which revealed chronic gastritis and acute ileitis. Labs were notable for anemia of 9.7 g/dL, thrombocytosis of 531 K/UL, and mild elevation of ESR 25 mm and CRP 34.2 mg/L. One month later he had persistent periumbilical abdominal pain, worsening weight loss, and labs significant for persistent anemia (8.8 g/dL), significant elevation of inflammatory markers (CRP 126 mg/L, ESR 92 mm, and elevated fecal calprotectin 1,129). CT of abdomen and pelvis showed marked distal ileal inflammation with extensive fistulization with 3 discrete fistulae (two to small bowel and one to the sigmoid). However, the degree of nodular wall thickening, somewhat aneurysmal dilation of the inflamed ileal loop, and sparing of the terminal ileum was radiologically described as atypical. He started metronidazole and infliximab induction for management of presumed Crohn’s disease. He described initial improvement with mild weight gain, decreased periumbilical abdominal pain, and improvement of inflammatory markers (ESR 43 mm and CRP 28 mg/L) within two weeks of initial infliximab dose. Two months later, recurrent symptoms required hospital admission with addition of steroids. Repeat imaging was unchanged and repeat fecal calprotectin remained elevated (>500). Due to failure of response to Infliximab he was switched to Ustekinumab.  He went home on metronidazole and a steroid taper and appeared to have improvement of abdominal pain and appetite. Two months later symptoms recurred with suprapubic abdominal pain and decreased appetite. Repeat CT showed interval increase in wall thickening of the terminal ileum with increased surrounding inflammation. He underwent resection of his terminal ileum and cecum with ostomy formation and was discharged days later following improved enteral intake. Unfortunately, histology of the resected bowel revealed mature B cell lymphoma.  He is currently readmitted for lymphoma directed therapy.
 

DISCUSSION:
Enteric fistulae in setting of weight loss, diarrhea, abdominal pain, elevated inflammatory markers, and anemia are classic findings of penetrating Crohn’s disease. However, this case points out the possibility of Crohn’s disease mimickers including lymphoma, which although rare can present with radiologic findings seen in Crohn’s disease including fistula formation stenosis, ulceration, and aneurysmal dilation.  It behooves us to consider alternative diagnoses if the presentation is atypical for IBD or symptoms fail to respond as expected.