IMAGE

OF THE

MONTH

Gastrointestinal Langerhans Cell Histiocytosis

FIGURE 1. Sigmoidoscopy showing scattered nodular, erythematous lesions extending from the rectum to the sigmoid colon.

FIGURE 2. High-power view (original magnification x40) showing a diffuse monomorphic infiltrate in the lamina propria, composed of mononuclear cells with irregular, elongated nuclei having grooves and folds characteristic of Langerhans cells.

A 5-month-old girl presented with 1 month of rectal bleeding, failure to thrive, and anemia. Appearing otherwise well, she was diagnosed as having cow’s-milk protein allergy. Despite change to elemental formula, symptoms persisted, with severe anemia (hematocrit 22.3%) and clinical findings worsening to include cervical lymphadenopathy, hepatosplenomegaly, and papular truncal rash, which had been diagnosed as eczema by a dermatologist. A sigmoidoscopy showed nodular, erythematous rectosigmoid lesions, some with central ulceration and evidence of recent bleeding (Fig. 1). Histology demonstrated Langerhans cell histiocytosis (LCH) having nodules of Langerhans cells, with irregular and clefted nuclei infiltrating the submucosa, staining positive for CD1a and CD68 (Fig. 2). LCH is a proliferative histiocytic disorder. Fewer than 1% of cases involve the gastrointestinal (GI) tract (1,2). GI LCH is associated with systemic disease and poor prognosis, with >50% mortality within 18 months of diagnosis (3–5). Although a rare presentation, with only 39 cases reported (13 of them with GI bleeding (6)), GI LCH should be considered in the differential diagnosis of infants with rectal bleeding and anemia unresponsive to amino acid–based formulae. Careful examination for systemic manifestations such as lymphadenopathy or cutaneous findings (eg, rash) can aid in diagnosis. Submitted by: Marcela M. Godoy, Melissa Fernandes, Gregory Mark Halenda, Golrokh Javid, and Peter D. Ngo Address correspondence and reprint requests to Peter D. Ngo, MD, Boston Children’s Hospital, Boston, MA (e-mail: [email protected]). The authors report no conflicts of interest. Submissions for the Image of the Month should include high-quality TIF endoscopic images of unusual or informative findings. In addition, 1 or 2 other associated photographs, such as radiological or pathological images, can be submitted. A brief description of no more than 200 words should accompany the images. Submissions are to be made online at www.jpgn.org, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

REFERENCES Yu RC, Attra A, Quinn CM, et al. Multisystem Langerhans’ cell histiocytosis with pancreatic involvement. Gut 1993;34:570–2. Hyams JS, Haswell JE, Gerber MA, et al. Colonic ulceration in histiocytosis X. J Pediatr Gastroenterol Nutr 1985;4:286–90. Singhi AD, Montgomery EA. Gastrointestinal tract Langerhans cell histiocytosis: a clinicopathologic study of 12 patients. Am J Surg Pathol 2011;35:305–10. Sabri M, Davie J, Orlando S, et al. Gastrointestinal presentation of Langerhans cell histiocytosis in a child with perianal skin tags: a case report. J Pediatr Gastroenterol Nutr 2004;39:564–6. 5. Hait E, Liang M, Degar B, et al. Gastrointestinal tract involvement in Langerhans cell histiocytosis: case report and literature review. Pediatrics 2006;118:e1593–9. 6. Yadav SP, Kharya G, Mohan N, et al. Langerhans cell histiocytosis with digestive tract involvement. Pediatr Blood Cancer 2010;55:748–53. 1. 2. 3. 4.

Copyright # 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition DOI: 10.1097/MPG.0000000000000027

JPGN



Volume 59, Number 2, August 2014

e17

Copyright 2014 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this article is prohibited.