Primary langerhans cell histiocytosis of the small bowel : report of three cases

Abstract EN

Background: Langerhans cell histiocytosis (LCH) represents a spectrum of disorders involving several tissues and organs which is characterized by proliferation of Langerhans histiocytes and various inflammatory cells.

The gastrointestinal tract may be involved in LCH, either as part of a generalized disease or as a separate primary entity.

Methods: Three cases of LCH with primary small bowel involvement were reviewed in the Department of Pathology at Al Assad University Hospital in Latakia in the period from l993-l999.

The identity of Langerhans cells was confirmed by immunohistochemical stains.

The patients were 2 men and l woman, aged 29, 44, and 68 years, each of whom presented with a history of chronic gastroenteritis and malabsorption syndrome.

One patient had an acute abdominal crisis associated with intestinal perforation.

Laparotomy was performed which revealed severe inflammation of the distal duodenum, the jejenum, and the proximal part of the ileum as well as many enlarged and congested mesenteric lymph nodes.

Results: Biopsy specimen obtained by endoscopy or from surgical resection of the small bowel (3rd case) revealed variable degrees of villous atrophy, dense inflammatory cell infiltrate of the lamina propria and submucosa by histiocytes, lymphocytes and a few eosinophils.

Some of the histiocytes showed the characteristic folded and grooved nuclei that identified them as Langerhans cells.

The majority of Langerhans cells expressed positive immunostains for CDla and Sl00.

Conclusion : This study included three cases of LCH involving the small bowel without systemic dissemination.

Because of the absence of recognized systemic manifestations, the histologic diagnosis was delaye.

Awareness of the unusual clinical presentation of LCH involving the small bowel may facilitate earlier accurate histologic diagnosis of this entity.