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)

We report a case with Langerhans cell histiocytosis appearing as an extra-pleural tumor. A 20-year-old man was transferred to our hospital because of right chest pain and fever. His chest X-ray showed an extra-pleural mass and chest CT scan showed a mass lesion with right 7th rib fracture. 67Gallium and bone scintigram showed uptake at the same site. We performed a CT-guided puncture biopsy. Pathological findings of the specimen showed diffuse proliferation of histiocytoid cells with some eosinophils. The histiocytes were positive for S-100 protein and CD1a on immunohistochemical stain. Langerhans cell histiocytosis was diagnosed. There was no other involvement of the disease except the rib. The tumor resolved only with smoking cessation and no recurrence was observed during the follow-up period. An association between smoking and progressions of the rib disease was suggested.
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PMID:[A case of Langerhans cell histiocytosis which resolved only on smoking cessation]. 1650 66

We report a rare case of coexisting pulmonary adenocarcinoma and Langerhans' cell histiocytosis (LCH) in a 78-year-old woman who did not smoke. During follow-up of diabetes mellitus, she had complained of chest pain and was found to have a nodular lesion in S9 of the left lower lobe, which was resected surgically. No abnormal laboratory findings were obtained. Before surgical resection, needle biopsy specimens confirmed the existence of adenocarcinoma. The resected tumor in the left lower lobe was 3.0 x 1.8 x 3.0 cm, and histologically both acinar and bronchioloalveolar cell subtypes of adenocarcinoma were found in cancer foci. In addition to pulmonary adenocarcinoma, Langerhans' cell proliferation associated with marked eosinophil infiltration was incidentally found in a small nodule, approximately 3 x 2 mm in size in the subpleural region. The Langerhans' cells contained interdigitated nuclei, exhibiting rather clear nucleoplasm and cytoplasm; they were positive for S-100 protein, CD1a, and also CD4. Massive eosinophil infiltration was found around the focus of Langerhans' cell proliferation. This nodule appeared to be LCH. The adenocarcinoma and LCH were adjacent, and cancer cells were infiltrated only in the peripheral parts of LCH. The coexistence of adenocarcinoma and LCH appeared to be incidental. The association of adenocarcinoma and LCH is rare, and only several reports of it can be found in the English literature.
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PMID:A rare case of coexistence of pulmonary adenocarcinoma with Langerhans' cell histiocytosis. 1880 45

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm arising from the antigen-presenting cells of the immune system. This disease usually involves the lymph nodes, and rarely, extranodal sites may be affected. The authors report a case of extranodal IDCS presenting in the pleura. A 32-yr-old man presented with progressive chest pain. Imaging studies showed diffuse pleural thickening with pleural effusion. Morphological and immunohistochemical analysis of an incisional biopsy of the pleura were consistent with a diagnosis of IDCS; tumor cells were positive for S100 and CD45, but negative for CD1a, CD21, CD35, B cell and T cell markers. The patient was administered chemotherapy, but died of progressive disease. Although its incidence is extremely rare, this case suggests that extranodal IDCS should be considered in the differential diagnosis of undifferentiated neoplasms and that immunohistochemical staining be performed using appropriate markers.
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PMID:Extranodal interdigitating dendritic cell sarcoma presenting in the pleura: a case report. 2128 27

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis most commonly characterized by symmetrical skeletal involvement and may present with pulmonary involvement leading to chronically progressive pulmonary symptoms. Characteristics on chest radiography include non-specific findings of diffuse interstitial and pleural thickening, micronodules, ground-glass opacities and parenchymal condensation as a result of infiltration by lipid-laden histiocytes. We present the case of a 50-year-old man with ECD presenting with acute pulmonary symptoms due to rupture of a large cystic lesion with resultant pneumothorax. He was brought by ambulance to our hospital, complaining of acute anterior chest pain and severe dyspnea. Chest radiography showed right-sided pneumothorax with a collapsed lung, a large, left-sided cystic lesion in the upper lung field and accentuated interstitial markings. Bullectomy and surgical biopsy were performed, demonstrating histologically histiocytic infiltrates that were strongly positive for CD68, but negative for S-100 protein and CD1a. Subsequent systemic examinations indicated widespread symmetrical skeletal involvement, leading to a definitive diagnosis of ECD.
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PMID:Erdheim-Chester disease presenting with pneumothorax. 2193 73

In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior.
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PMID:A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female. 2604 67