Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Protein-losing enteropathy was observed in two children with Langerhans' cell histiocytosis (LCH). One patient was an infant with congenital cutaneous lesions; the second child had sigmoid and lymph node infiltration. Electron microscopy and immunohistochemistry confirmed, in both, infiltration of duodenum, skin, and liver by LCH. Gastrointestinal involvement by LCH seldom produces prominent clinical manifestations but indicates widespread multisystem disease. Immunohistochemical and/or ultrastructural features allow definitive diagnosis from mucosal biopsy specimens. Review of the literature of gastrointestinal infiltration by LCH emphasizes its poor prognosis, especially when associated with organ dysfunction.
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PMID:Digestive tract involvement with exudative enteropathy in Langerhans cell histiocytosis. 140 50

A case of Langerhans cell histiocytosis is reported in a neonate. Intestinal involvement was especially diffuse and severe, presenting as a protein-losing enteropathy secondary to massive mucosal infiltration by histiocytic cells. The infant died at the age of 3 1/2 months despite therapy with corticosteroids and vinblastine then etoposide and interferon. Such an outcome confirmed the severity of forms with neonatal onset and digestive involvement.
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PMID:[A rare cause of neonatal exudative enteropathy: congenital Langerhans cell histiocytosis (histiocytosis X)]. 161 Feb 78

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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PMID:Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy. 1046 85

We report unusual findings in two patients with Langerhans' cell histiocytosis (LCH), namely a cervical mass lesion with extensive destruction of the posterior elements of a cervical vertebra and gastrointestinal lesions as part of multisystem involvement. The aim of our report is threefold. Firstly, LCH can be responsible for osteolysis of the vertebral posterior arches, with or without involvement of the vertebral body, and should be included in the differential diagnoses of cervical mass lesions. Secondly, in a patient with confirmed LCH and additional protein-losing enteropathy, gastrointestinal involvement should be considered as a possibility since it is an important factor for establishing prognosis. Thirdly, LCH lesions can be very extensive and yet have a good response to therapy, whereas less spectacular lesions may not respond or respond only partially to therapy. Thus, an important factor in establishing prognosis is the presence of multisystem involvement at diagnosis, regardless of the extent of a lesion at a particular site.
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PMID:Unusual findings in two cases of Langerhans' cell histiocytosis. 1075 62

Protein-losing enteropathy (PLE) is frequently complicated in patients with gastrointestinal tract-involved Langerhans cell histiocytosis (LCH); however, LCH per se is not generally included in the list of diseases that cause PLE. We report here a case of infantile PLE that presented with continuous diarrhea at the onset of LCH. She was initially diagnosed as having allergic gastroenteropathy and, thus, received intravenous prednisolone, which was thought to have induced immunodeficiency and consequently resulted in life-threatening cytomegalovirus-associated hemophagocytic syndrome and disseminated intravascular coagulation. Because chemotherapy for hemophagocytic syndrome was transiently effective for underlying LCH as well, the diagnosis of LCH was delayed until its recurrence. Gastrointestinal tract-involved LCH, a rare but highly fatal disease, should be considered for infants with refractory gastrointestinal symptoms, especially for those with PLE; endoscopic biopsy is strongly recommended for immediate diagnosis.
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PMID:Protein-losing enteropathy caused by gastrointestinal tract-involved Langerhans cell histiocytosis. 2008 19

Gastrointestinal tract (GIT) involvement in Langerhans cell histiocytosis (LCH) is not commonly described. We present two children presenting with GIT involvement with LCH, one successfully treated on standard protocol and other being treated on a protocol for relapsed disease. A review of literature showed almost 95% children were less than 2 years of age and 62% were females. Vomiting, abdominal pain, constipation, intractable diarrhea, malabsorption, bloody stools, protein-losing enteropathy, and even intestinal perforation are some of the reported symptoms. More than 50% patients died within 18 months from diagnosis.
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PMID:Langerhans cell histiocytosis with digestive tract involvement. 2053 29

Gastrointestinal (GI) tract involvement in Langerhans cell histiocytosis (LCH) is extremely rare. Langerhans cell histiocytosis with GI tract involvement (GI-LCH) is frequently associated with multi-system disease, and usually presents with severe systemic symptoms, such as protein-losing enteropathy (PLE). Although the GI tract is not included among the organs at risk, the prognosis of GI-LCH is poor, and no effective chemotherapeutic regimen has been identified. Here, we report an infant case of primary refractory GI-LCH with PLE that showed marked improvement in response to 2-chlorodeoxyadenosine (2-CdA) therapy with no severe adverse events, even under conditions of deteriorating general health. The present findings indicate that 2-CdA may be effective for refractory GI-LCH with PLE. Further studies are warranted to determine the optimal therapeutic strategies for GI-LCH with PLE.
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PMID:Successful treatment with 2-chlorodeoxyadenosine of refractory pediatric Langerhans cell histiocytosis with initial involvement of the gastrointestinal tract. 3135 Jun 65