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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Symptoms of gastrointestinal disease are variable in Langerhans' cell histiocytosis (LCH). The incidence of gastrointestinal involvement is estimated to be approximately 5% in disseminated LCH. This report focuses on a 10.5 months old female infant who suffered from relapsing diarrhea and intermittent anal blood loss since the second week of life and from weight loss later in the course of disease. Definite diagnosis of LCH was not established before the patient developed additional characteristic symptoms. Analysis of large series of patients and a retrospective histopathological study reveals that an involvement of the gastrointestinal tract must be anticipated in about 50% of all cases with disseminated LCH.
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PMID:[Gastrointestinal symptoms in disseminated Langerhans cell histiocytosis. Case report and review of the literature]. 207 67

The gastrointestinal tract may be involved in Langerhans' cell histiocytosis, either as part of a generalized disease or as a separate primary entity. Three patients with gastrointestinal involvement are described. One patient presented with a history of prolonged vomiting and diarrhoea with blood and mucus. In this case the diagnosis was based on colonic biopsies. Gastrointestinal tract involvement and its investigation in the diagnosis of Langerhans' cell histiocytosis are discussed.
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PMID:Gastrointestinal involvement in Langerhans' cell histiocytosis (Histiocytosis X): a clinical report of three cases. 217 34

We report two patients with biopsy-proven involvement of the gastrointestinal tract by Langerhans cell histiocytosis (LCH). The two patients were infants with congenital cutaneous lesions and bloody diarrhea beginning at 1 or 2 wk of age. Rectal biopsy specimens showed a mucosal infiltrate of typical Langerhans cells that focally invaded and destroyed the crypt epithelium. Gastrointestinal involvement by LCH is unusual and only rarely has represented a prominent clinical manifestation. In most cases, such involvement has been an indicator of widespread multisystemic disease. Its distinctive morphologic and immunohistochemical features allow LCH to be distinguished from other histiocytic infiltrations found in mucosal biopsy specimens.
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PMID:Gastrointestinal involvement in Langerhans cell histiocytosis (histiocytosis X): diagnosis by rectal biopsy. 218 12

A chronic diarrhea accompanied by vomiting and weight loss was the major symptom in a one-year-old infant during a fulminant course of histiocytosis X. The diagnosis was suggested by the radiologic evidence of alternating dilated and stenotic segments in the small and large bowel as well by a massive intestinal protein loss and the presence of histiocytes in the mucosa and submucosa of the rectum. The histological picture of enlarged mesenteric lymph nodes, obtained during a diagnostic laparotomy, confirmed the diagnosis.
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PMID:[Chronic diarrhea as the main symptom of histiocytosis X]. 278 13

The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
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PMID:New insight into the causes of immunodeficiency disorders. 638 1

A case of benign regressing histiocytosis of the liver is reported. The patient, an adult male, presented with fever and diarrhoea and on abdominal echography multiple nodules were present in the liver. Histologically and immunohistochemically the lesions had features of Langerhans' cell histiocytosis. The nodules regressed within four months, without therapy, and the patient is free of disease 29 months after presentation.
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PMID:Benign regressing histiocytosis of the liver. 760 26

A boy with skin eruptions resembling varicella and specific for Langerhans cell histiocytosis (LCH) is reported. At his initial visit when he was four months old, vesiculopustular lesions were present over the entire body; these had first appeared on the third day post partus. Histopathological, immunohistochemical, and electron microscopical examination confirmed the Langerhans cell phenotype and Birbeck granules in the responsible cells. He also had hydronephrosis, recurrent fever, and cutaneous bacterial infections. His parents refused further medical treatment and he died of diarrhea with cachexia about two years later. LCH may present diagnostic difficulties by manifesting as a skin eruption which resembles varicella.
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PMID:Langerhans cell histiocytosis presenting as a varicelliform eruption over the entire skin. 801 75

We present a case of a patient with common variable immune deficiency presenting as the Letterer-Siwe syndrome (disseminated Langerhans cell histiocytosis). To our knowledge, this is the only known patient with this association. The clinical presentation was chronic diarrhea, weight loss, recurrent infections, hepatosplenomegaly, and interstitial pneumonitis. Laboratory evaluation revealed evidence of immunodeficiency, with agammaglobulinemia and diminished number and function of T cells. The diagnosis of Langerhans cell histiocytosis was confirmed by electron microscopic examination of the lung biopsy specimen demonstrating Birbeck granules in the cells of the infiltrate. It is known that patients with the combined immunodeficiency syndrome may present as disseminated Langerhans cell histiocytosis, and the case presented demonstrates that patients with common variable immune deficiency may similarly present. It is advisable that patients newly diagnosed with Langerhans cell histiocytosis be evaluated to screen for immunodeficiency. Conversely, patients presenting with combined immunodeficiency or common variable immune deficiency may display features of disseminated Langerhans cell histiocytosis. These associations must be considered in newly diagnosed immunodeficient patients.
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PMID:A 13-month-old child with chronic diarrhea, weight loss, and tachypnea. 821 98

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

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


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