Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

Symptomatic involvement of the gastrointestinal (GI) tract as a prominent s ymptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in le ss than 1 to 5% of all cases, even when the disease is in its disseminated form, Up to now, there have been reports of 18 cases of LCK with GI manifes tations, including our 2 cases, with diarrhea (77.7%), protein-losing enter opathy (33.3%) and bloody stool being the most frequent findings. The autho rs present two patients with severe diarrhea and refractory hypoalbuminemia , and with the protein-losing enteropathy documented by Cr-51-labeled album in studies. A review of the literature indicated that the presence of GI sy mptoms is often associated with systemic disease as well as with poor progn osis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, an d their utilization in the cases reviewed here permitted diagnoses in 6 chi ldren, as well as improved therapeutic management.