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BMJ Case Rep. 2010; 2010: bcr12.2008.1343.
Published online Jan 13, 2010. doi:  10.1136/bcr.12.2008.1343
PMCID: PMC3029650
Rare disease
Florid reactive lymphoid hyperplasia of terminal ileum
Venkatesh Kanakala,1 Peter Birch,2 and Ramesh Kasaraneni1
1North Tyneside General Hospital, General Surgery, Rake Lane, North Shields NE29 8NH, UK
2North Tyneside General Hospital, Histopathology, Rake Lane, North Shields NE3 1TW, UK
Venkatesh Kanakala, ven.kanakala/at/gmail.com
Abstract
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.
Background
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.
Case presentation
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 1
Figure 1
Reactive lymphoid hyperplasia in small intestinal mucosa.
Figure 2
Figure 2
Distension and filling of submucosal lymphatics by small lymphocytes.
Figure 3 shows a plain abdominal x-ray of the patient, showing normal gas pattern with no obvious radiological abnormality.
Figure 3
Figure 3
Plain abdominal x-ray, showing normal gas pattern with no obvious radiological abnormality.
Differential diagnosis
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.
Treatment
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.
Outcome and follow-up
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.
Learning points
  • Florid lymphoid hyperplasia can present to surgeons as acute appendicitis.
  • At operation it might look like Crohn’s disease
  • Local surgical resection is curative.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
1. Briquet Cruveilher’s atlas, vol. II 1835–1842. 34.
2. Marina-Fiol C.. Exploracio’n del ileon terminal. Rev Clin Esp 1941; 3: 97–105.
3. Dorazio RA, Whelan TJ., Jr Lymphoid hyperplasia of the terminal ileum associated with familial polyposis coli. Ann Surg 1970; 171: 300–2. [PubMed]
4. Swartley RN, Stayman JW., Jr Lymphoid hyperplasia of the intestinal tract requiring surgical intervention. Ann Surg 1962; 155: 238–40. [PubMed]
5. Stueland DT, Binkley NC, Magnin GE. Symptomatic benign lymphoid hyperplasia of the terminal ileum in an adult. Am J Gastroenterol 1980; 74: 522–4. [PubMed]
6. Charlesworth D, Fox H, Mainwaring AR. Benign lymphoid hyperplasia of the terminal ileum. Am J Gastroenterol 1970; 53: 579–84. [PubMed]
7. Rubin A, Isaacson PG. Florid reactive lymphoid hyperplasia of the terminal ileum in adults: a condition bearing a close resemblance to low-grade malignant lymphoma. Histopathology 1990; 17: 19–26. [PubMed]
8. Ganly I, Shouler PJ. Focal lymphoid hyperplasia of the terminal ileum mimicking Crohn’s disease. Br J Clin Pract 1996; 50: 348–9. [PubMed]
9. Yunis EJ, Hashida Y. Electron microscopic demonstration of adenovirus in appendix vermiformis in a case of ileocecal intussusception. Pediatrics 1973; 51: 566–70. [PubMed]
10. Capitanio MA, Kirkpatrick JA. Lymphoid hyperplasia of the colon in children. Roentgen observations. Radiology 1970; 94: 323–7. [PubMed]
11. Thomford NR, Greenberger NJ. Lymphoid polyps of the ileum associated with Gardner’s syndrome. Arch Surg 1968; 96: 289–91. [PubMed]
12. Fieber SS, Schaefer HJ. Lymphoid hyperplasia of the terminal ileum—a clinical entity? Gastroenterology 1966; 50: 83–98. [PubMed]
13. Danis RK. Lymphoid hyperplasia of the ileum—always benign disease? Am J Dis Child 1974; 127: 656–62. [PubMed]
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