Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

Abstract

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 Cr 51 -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.

Protein-Losing Enteropathy due to Intestinal and Colonic Involvement With Langerhans Cell Histiocytosis and Review of the Literature

“…High risk organs include hematopoietic cells, liver, spleen, and lungs; involvement of these organs portends a worse prognosis. 4,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] Children with LCH of the bowel present early in life with a mean (±standard deviation) age at diagnosis of 8.6 (±8.2) months ( Table 1). 6,7 We reviewed the clinical data of 36 children with LCH of the bowel identified in the English literature.…”

Intestinal Langerhans Cell Histiocytosis-like Lesion in an Adult Presented with Diverticulitis: A Reactive or Neoplastic Condition?

“…However, this is most often described in children with multiorgan or disseminated disease [1][2][3][4][5]. Furthermore, the association of LCH-like lesion with diverticulitis has not been reported so far in the English literature.…”

“…Furthermore, the association of LCH-like lesion with diverticulitis has not been reported so far in the English literature. In most cases, LCH of the gut has been presented with protein-losing enteropathy and diarrhea [2][3][4][5]. However, when systemic disease is present; several sites such as skin, bones, liver, spleen, lungs and lymph nodes are usually affected [6].…”

“…In most cases, such condition has been regarded as an indicator of a disseminated disease [1,2]. Protein-losing enteropathy and diarrhea (sometimes bloody) are reported to be the main clinical findings of intestinal LCH [2][3][4][5].…”

Clinical Quiz

“…It is probably underdiagnosed, as gastrointestinal tract disease can be clinically silent. Digestive involvement seems to confer a worse prognosis to the disease, probably because of refractory hypoalbuminemia (3,4).…”