A Rare Triad of Crohn’s Disease, Eosinophilic Enteritis, and Castleman’s Disease

Excerpt

A 25-year-old man presented with recurrent abdominal pain for 2 years. He had history of severe anemia 1 year back requiring blood transfusion. The patient had chronic urticaria. Blood count revealed high eosinophil count (10%). Upper gastrointestinal endoscopy and colonoscopy were unremarkable. Contrast-enhanced computed tomography of the abdomen had revealed circumferential hypodense wall thickening of the mid-ileal loops with proximal bowel dilatation and few air-fluid levels (Fig. 1). Fecal calprotectin level was > 3000 mg/kg. The patient was diagnosed with inflammatory bowel disease and started on immunosuppressive drugs. However, the symptoms persisted. At surgery, a short-segment non-passable stricture was found in the proximal ileum. The involved segment of the bowel was resected. The postoperative recovery was uneventful. On histopathology, there was moderate to dense mixed inflammatory infiltrate in the lamina propria and muscular layer with fairly increased number of eosinophils suggestive of eosinophilic enteritis (Fig. 2). In addition, small noncaseating granulomas were present indicating Crohn’s disease (Fig. 2). The histology of mesenteric lymph nodes revealed hyaline-vascular type Castleman’s disease characterized by onion skin appearance of the mantle zone with IgD positive mantle cells and CD23 stained follicular dendritic network (Fig. 2). Based on the histological diagnosis, he was started on low-dose azathioprine to prevent recurrence. During the follow-up period of 5 months, he had one episode of subacute intestinal obstruction which was managed conservatively. Currently, he is receiving steroids along with azathioprine and is symptom-free.

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