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)

A 61-year-old woman presented with circumscribed eczematous eruptions with maceration, erosions and patchy infiltration in the perineum and inframammary regions. A diagnosis of eosinophilic granuloma (cutaneous histiocytosis X) was established. T lymphocytes from a skin biopsy were grown in vitro for three weeks after which chromosomal studies revealed a break or gap at chromosome 16q22 in 15% of the lymphocytes. The addition of alpha-interferon increased the percentage of affected cells to 28%. T lymphocytes from the patient's blood did not show the defect. The biological significance of the chromosomal defect is uncertain. It has been described before in healthy persons, malignant lymphoma, cold urticaria and IgA deficiency, and mental retardation. It has not been seen in patients with eosinophilic granuloma.
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PMID:Eosinophilic granuloma associated with a 16q22 chromosomal defect of cutaneous T lymphocytes. 608 32

The term histiocytosis X (HX) refers to a spectrum of disorders varying from unifocal eosinophilic granuloma (UEG), multifocal eosinophilic granuloma (MEG), to the Abt-Letterer-Siwe syndrome. In a series of 16 patients with different types of HX and skeletal lesions, whole body bone scintigraphy was performed at the time of diagnosis and during follow up. Results were compared with radiographic findings. In patients with MEG with or without extra-skeletal dissemination bone scintigraphy revealed cold spots or hot spots, but half of the lesions were not visualised scintigraphically, resulting in false negative scans. In UEG the lesions were visualised as areas of increased uptake or as a cold spot with increased uptake at its borders. No false negative scans were encountered. The reliability of skeletal scintigraphy in patients with HX seems to depend on the type of the disorder: in UEG bone scintigraphy is a safe procedure. In MEG false negative bone scans have to be expected, and radiography is superior.
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PMID:Radionuclide bone scintigraphy in patients with histiocytosis X. 660 76

Thyroid involvement by Langerhans cell histiocytosis is rare. We report the case of a ten-year old boy who presented with a 5 cm goitre. He was treated for diabetes insipidus 14 months before. Thyroid isotopic scan showed hypoactivity of right lobe and revealed a cold left nodule. The patient was treated by right lobo-isthmectomy with adjuvant corticotherapy and chemotherapy (vinblastine). He is well with 12 months follow-up. Microscopic analysis demonstrated a diffuse infiltrate of thyroid parenchyma by sheets of CD1a positive Langerhans cells associated with lymphocyte foci. This case is remarkable by the abundance of Langerhans cells and scarcity of eosinophils. The diagnosis of thyroid langerhans cell histiocytosis should not be ignored in both children and adult patients.
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PMID:[Langerhans cell histiocytois of the thyroid: a rare disease not to be ignored]. 1193 98