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Florid lymphoid hyperplasia in the terminal ileum can present to surgeons as an acute abdominal pain. Only few cases were reported in the literature. Our case illustrates that a rare case of florid lymphoid hyperplasia can present to surgeons as acute appendicitis. During the operation the gross appearance may mimic Crohn’s disease. A limited resection is sufficient to clinch the diagnosis of florid lymphoid hyperplasia / Crohn’s disease. In florid lymphoid hyperplasia limited resection may be curative.
Florid lymphoid hyperplasia in the terminal ileum is a rare condition with an unknown aetiology. This condition was first reported by Briquet in 18381 and later it was investigated radiologically by Marina-Fiol and Rof-Carballo2 in 1941 and given a new name of “enteritis follicularis”. Lymphoid hyperplasia may occur anywhere in the gastrointestinal tract, from the base of tongue to the anus, but is more common at the terminal ileum and rectum.3,4 The common presentation is abdominal pain with or without loose stools or gastrointestinal bleeding. Very few cases have been reported from UK. Here we report our case which presented as acute appendicitis.
A 60-year-old female patient was admitted to the accident and emergency department with a history of pain in the right iliac fossa for 1 week and a worsening pain over the past few days. She had no other gastrointestinal (GI) symptoms such as nausea/vomiting, altered bowel habits, bleeding per rectum, or weight loss. Her past medical/surgical history included arthritis, cholecystectomy, hysterectomy, and vaginal and rectal prolapse repair. She was taking methotrexate for her osteoarthritis. All the vital parameters were normal. On examination there was minimal abdominal distension with severe tenderness in the right iliac fossa.
Blood investigations revealed increased inflammatory markers such as white blood cell count (13.6×109/l), neutrophils (10.4×109/l) and C reactive protein (35 IU) with normal liver function tests.
The histopathology after operation confirmed florid reactive lymphoid hyperplasia (fig 1). Grossly, the specimen comprised 170 mm of terminal ileum, ileocaecal valve and caecum of 70 mm in length. The mucosal surface of the small bowel in the area of ileocaecal valve and terminal ileum appeared irregular with longitudinal ulcerations. On microscopic examination, there was pronounced lymphoid hyperplasia of the terminal ileum within an area of Payer’s patch, with increased small lymphoid cells and reactive lymphoid follicles. The mucosal surface was focally ulcerated and there were transmural lymphoid aggregates. Eosinophils and occasional neutrophils were also present within the small bowel wall and on the serosal surface. Submucosal lymphatic tissue was distended and filled with lymphocytes (fig 2). Immunohistochemistry showed a mixed kappa and lambda light chain population together with T and B lymphocytes. The mesenteric lymph nodes showed reactive hyperplasia. Both proximal and distal resection margins were not involved and free of disease.
Figure 3 shows a plain abdominal x-ray of the patient, showing normal gas pattern with no obvious radiological abnormality.
This condition may present to a clinician with clinical features suggestive of acute appendicitis, intestinal obstruction, Crohn’s disease or malignant tumour of the small/large bowel.
A clinical diagnosis of acute appendicitis was made and a diagnostic laparoscopy was performed with an intention to perform an appendectomy. During the operation it was observed that the appendix was grossly normal but the terminal ileum was notably thickened with associated mesenteric lymphadenopathy. The procedure was converted to a laparotomy. During the operation there was a caecal mass and thickened terminal ileum and its mesentery. This was felt to possibly represent Crohn’s disease and a limited ileocaecal resection followed by ileocolic anastomosis was performed.
The patient recovered from the procedure well and was discharged home without any complications, with a further clinical follow-up after 6 months.
Our patient presented with symptoms and signs of acute appendicitis with raised inflammatory markers. Several studies have emphasised the importance of raised inflammatory markers, when a diagnostic dilemma of appendicitis arises. Hence we decided to do a diagnostic laparoscopy with an intention to perform an appendectomy.
Lymphoid hyperplasia is very common in children but is very uncommon in adults.5 The aetiology of florid lymphoid hyperplasia is hypothesised to be the result of an antigenic response,6 Yersinia infection7,8 Adenovirus infection9 and Shigella.10 In our case, no specific source of infection was identified.
Rubin et al illustrated that terminal ileal lymphoid hyperplasia can be divided into childhood (common) and adult (rare) form.7 The adult form is difficult to distinguish from low grade lymphoma, but can only be differentiated by the absence of light chain restriction.7,8 In our case we demonstrated both kappa and lambda light chains on immunohistochemical staining. Though there are a few case reports of association with other systemic diseases such as multiple intestinal polyposis,3 Gardner syndrome,11 and malignant lymphoma,12 these associations were noted to occur only in children <10 years of age.13 The malignant potential of this condition in adults is still unclear. Our case did not show any evidence of malignancy. Larger case series are required to establish the aetiopathogenesis and malignant potential of this condition. It can be misdiagnosed as acute appendicitis or Crohn’s disease at surgery, as happened in our case.
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.