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)

Lymph nodes contain nonlymphoid accessory cells including follicular dendritic cells (FDCs), interdigitating dendritic cells (IDCs) and fibroblastic reticular cells (FBRCs). Neoplasms derived from FDCs are uncommon, and those of IDC origin are even more rare. We report the clinicopathologic features of 11 reticulum cell neoplasms, including 2 of FBRC origin. There were seven male patients and four female patients ranging in age from 13 to 73 years. All cases involved lymph nodes (cervical or supraclavicular-6 cases), (abdominal--2 cases), epitrochlear (1 case); two had more than one site of involvement (cervical lymph node and mediastinum--1 case, cervical and abdominal lymph nodes--1 case). One case of FDC tumor had concomitant Castleman's disease, plasma cell variant. Each neoplasm showed similar histology with oval-to-spindle-shaped cells in a storiform or fascicular pattern. Based on immunophenotypic findings, the neoplasms were classified as FDC (five cases), IDC (two cases), FBRC (three cases), and reticulum cell neoplasm, not otherwise specified (one case). The FDC tumors showed immunoreactivity for CD21 or CD35, vimentin, and CD68. The IDC tumors showed strong positivity for S-100 protein and variable positivity for CD68 and CD1a. The cases derived from FBRCs were positive for vimentin, desmin, and smooth-muscle actin. The neoplasm classified as reticulum cell neoplasm, not otherwise specified had similar morphologic features but showed only equivocal positivity for CD68 and vimentin. Follow-up was available for 9 of 11 (82%) cases with a mean of 3.5 years. Four of five patients with FDC tumors were alive with disease when last seen; the fifth is alive and well with no evidence of disease at 4-year follow-up. One patient with IDC tumor had a recurrence in a different nodal site. Two patients with FBRC tumor were disease free at follow-up of 2 years and 8 years, respectively. The patient with reticulum cell neoplasm, not otherwise specified, was alive and disease free 8 years after diagnosis.
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PMID:Reticulum cell neoplasms of lymph nodes: a clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells. 973 36

We describe an 18-year-old woman with several month's history of a 12 x 7 mm palpable mammary nodule, that was hypoechoic, with regular margins and vascularization areas by ultrasound. A fibroadenoma was hypothesized (American College of Radiology BI-RADS 3). A 14 G needle biopsy was performed, showing a LC proliferation suspected for LCH of a lymph node, with florid dermatopathic lymphadenopathy in differential diagnosis. The multidisciplinary team of the breast clinic decided to perform a lumpectomy and a diagnosis of LCH involving an intra-mammary lymph node was made. Langerhans cells (LC) are dendritic cells characterized by grooved nuclei, irregular nuclear contours, and abundant cytoplasm, that normally reside in the skin and mucosal surfaces. They were positive for CD1a, langerin/CD207, and S100 by immunohistochemistry. Langerhans cell histiocytosis (LCH) is a clonal proliferation of histiocytes that is thought to be neoplastic in most cases. Reactive LC can be distinguished from LCH by cyclin D1 immunostaining, which is positive only in LCH. About 50% of cases have BRAF V600E mutations. The revised classification of histiocytes divides LCH in subtypes: LCH SS (single system), LCH lung positive, LCH Multiple System/Risk Organ negative and LCH Multiple System/Risk Organ positive. Localized disease can progress to multisystem involvement. The diagnosis of LCH is based on clinical and radiological findings in combination with histopathological, immunophenotypic or ultrastructural analyses identifying tissue infiltration by LC. It is recommended that biopsy confirmation of suspected LCH be performed in all cases. Lymph nodes may be the only site of disease or a part of multisystem involvement by LCH. The histologic differential diagnosis is discussed.
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PMID:Langerhans cell histiocytosis of an intra-mammary lymph node in an 18-year-old woman. 3220 40