Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

Brazilian Journal of Medical and Biological Research

T.m. Santos-machado¹  L.m. Cristófani¹  M.t.a. Almeida¹  P.t. Maluf¹  P.a. Costa¹  M.a. Pereira¹  J.l.b.c. Brito¹  V. Odone-filho¹ 

[1] ,Universidade de São PauloSão Paulo SP ,Brasil

关键词: Langerhans' cell histiocytosis;    histiocytosis X;    gastrointestinal involvement;    protein-losing enteropathy;    hypoalbuminemia;    Cr51-labeled albumin test;   

DOI  :  10.1590/S0100-879X1999000900007

来源: SciELO

【 摘 要 】

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.

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