Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis involving bones and multiple organs. Its clinical course can vary, from an asymptomatic state to a fatal disease, with renal involvement being a common cause of death. A 41-year-old man presented with a 10-month history of bilateral lower limb pain. Left perirenal soft-tissue infiltration had been found incidentally two years earlier. No progression of the lesion or deterioration of renal function was observed for a period of two years. At admission, plain radiography and magnetic resonance imaging of the patient's lower limbs showed patchy osteosclerosis. Biopsy of the tibia revealed histiocytic infiltration, which was found to be positive for CD68 and negative for CD1a. This report describes an unusual case of Erdheim-Chester disease involving a stationary course of disease with no specific treatment for a long period of time.
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PMID:A case of erdheim-chester disease with asymptomatic renal involvement. 2280 54

Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause, which encompasses a set of disorders of multiple organs with various clinical presentations that share the common characteristic of sizeable Langerhans cell infiltration. The clinical spectrum of LCH ranges from solitary bone lesions to involvement of other organs. lung involvement in LCH may be part of a multisystem disease, which almost exclusively occurs in adult smokers, while it is overlooked or misdiagnosed in the majority of non-smokers. High-resolution computed tomography (HRCT) of the chest is crucial for diagnosis; however, the treatment and prognosis of this disease have not been clearly determined. We herein present the case of a non-smoking adult patient who presented with lower limb pain and was diagnosed via biopsy with lch with multisystem involvement, including the bone and lungs. Lytic lesions in the corpus of the sacroiliac joint, sacrum, acetabulum and femoral head by a soft tissue mass were observed on diagnostic CT. In addition, chest HRCT revealed multiple cysts in the bilateral lungs, predominantly in the upper lobes. The final diagnosis of LCH was confirmed by histopathological examination and immunohistochemical staining for CD1a and S-100. Corticosteroid treatment alleviated lower limb pain and improved the patient's quality of life; thus, corticosteroids may be considered as a potential treatment option for patients with LCH.
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PMID:Bone Langerhans cell histiocytosis with pulmonary involvement in an adult non-smoker: A case report and brief review of the literature. 2812 31