Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy.
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.
Santos-Machado
T M
TM
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Cristófani
L M
LM
Almeida
M T
MT
Maluf
P T
PT
Costa
P A
PA
Pereira
M A
MA
Brito
J L
JL
Odone-Filho
V
V
D001706:Biopsy; D002675:Child, Preschool; D004064:Digestive System / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D015614:Histiocytosis / Q000473:pathology*; D006801:Humans; D006994:Hypoaldosteronism / Q000150:complications; D008297:Male; D011504:Protein-Losing Enteropathies / Q000473:pathology*