Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

• Autor(a) principal: Santos-Machado,T.M.
• Data de Publicação: 1999
• Outros Autores: Cristófani,L.M., Almeida,M.T.A., Maluf,P.T., Costa,P.A., Pereira,M.A., Brito,J.L.B.C., Odone-Filho,V.
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Brazilian Journal of Medical and Biological Research
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1999000900007
• topic: Langerhans' cell histiocytosis; histiocytosis X; gastrointestinal involvement; protein-losing enteropathy; hypoalbuminemia; Cr51-labeled albumin test

Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less 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 LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%), protein-losing enteropathy (33.3%) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.