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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The cutaneous histiocytoses are best divided in the Langerhans' cell histiocytoses and non-Langerhans' cell histiocytoses. In the former group, the cells react with S100 and CD1a antibodies, while in the latter group they express a variety of macrophage markers. Xanthogranuloma is a frequent childhood tumor and the only common non-Langerhans' cell histiocytosis. Xanthogranulomas contain a mixture of several different types of histiocytes that also appear in more pure forms as both solitary tumors and disseminated processes. The varying histiocyte morphology provides a unifying concept for non-Langerhans' cell histiocytoses.
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PMID:The non-Langerhans' cell histiocytoses in childhood. 888 34

Twenty-nine cases of histiocytic neoplasms, some resembling juvenile xanthogranuloma (JXG) and others resembling reticulohistiocytoma (RH), were evaluated. Immunohistochemical stains were performed. In this series, seven cases were identified that expressed S-100 protein positive cells. The S-100 positive cells were predominantly large mononuclear and multinucleated histiocytes with eosinophilic cytoplasm, but also in some cases xanthomatous cells and Touton giant cells. These cells also expressed a positive reaction for vimentin, KP-1, and Factor XIIIa. There was no reactivity observed for monoclonal antibody 010(CD1a). A positive reaction for S-100 protein is conventionally accepted as a useful differentiating feature between histiocytosis X and non-X histiocytosis such as JXG and RH. The conflicting results of the immunohistochemical stains in the lesions we studied could be potential pitfalls in diagnosing histiocytic neoplasms.
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PMID:Unusual expression of S-100 protein in histiocytic neoplasms. 955 Mar 10

Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over Langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.
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PMID:Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? 1069 21

We report three children who had multisystem Langerhans cell histiocytosis (LCH) with cutaneous involvement and subsequently developed juvenile xanthogranuloma (JXG). JXG appeared 3--6 years after the initial manifestation of LCH. JXG lesions, which presented as yellowish papules, revealed typical Touton giant cells and were factor XIIIa positive but S100 and CD1a negative. Non-LCH histiocyte disorders, such as JXG, are known to occur as a reaction to a variety of external stimuli such as infection and trauma. It is therefore conceivable that the inflammatory reaction associated with LCH may have precipitated the development of JXG in our patients. Alternatively, one could speculate that this association might be due to a common histogenetic precursor of the cell types involved.
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PMID:Juvenile xanthogranuloma as a sequel to Langerhans cell histiocytosis: a report of three cases. 1148 23

Systemic form of juvenile xanthogranuloma with involvement of liver and bone marrow is reported in a 2-month-old female infant who presented with hepatosplenomegaly, severe anemia, and thrombocytopenia. There was no skin lesion, nor bone lesion. The enlarged liver has generalized yellowish spots. The diagnosis of juvenile xanthogranuloma was made by pathologic findings of marrow and portal tract infiltration by S-100 negative, CD1a negative, CD68 positive, and Factor XIIIa positive large pale to foamy histiocytes with Touton giant cells, and lack of Langerhans cell granule by electron microscopic examination. The patient was treated with Vinblastine and Etoposide, and experienced slow and gradual disease regression in one year. To the best of knowledge, this is the first documented case of bone marrow involvement in systemic juvenile xanthogranuloma.
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PMID:Systemic form of juvenile xanthogranuloma: report of a case with liver and bone marrow involvement. 1563 May 37

Juvenile xanthogranuloma (JXG) is a benign histiocytic disorder of infants and childhood. Approximately 15% of cases occur in adults. Adult JXG characteristically affect patients in their 20s and 30s; however, about 5% of patients are older than 60 years. Adult JXGs rarely regress spontaneously, and reports of concomitant extracutaneous lesions are rare. Herein, we report an exceptional case of adult xanthogranuloma in a 74-year-old woman who presented with ipsilateral breast masses and also found to have prior cutaneous lesions. This is the first reported case of cutaneous and extracutaneous adult JXG where the latter manifested in the breast as a spindle cell xanthogranuloma. Histologically, the lesion was composed predominantly of spindle cells with associated multinucleated giant cells and a chronic inflammatory cell infiltrate. Spindle cells were immunoreactive for various histiocytic markers and negative for cytokeratins, S-100, CD34, factor XIIIa, and CD1a. In the breast, the morphologic features of JXG evoked several entities in the differential diagnosis, including spindle cell metaplastic carcinoma, inflammatory pseudotumor, fibromatosis, myofibroblastoma, and phyllodes tumor. With the aid of immunohistochemical stains and appropriate clinical history, the correct diagnosis of extracutaneous adult JXG manifesting as a spindle cell xanthogranuloma can be made.
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PMID:Mammary presentation of adult-type "juvenile" xanthogranuloma. 1589 51

A 3-year-old Thai boy suffered from two histiocytoses, Rosai-Dorfman disease (RDD) and juvenile xanthogranuloma (JXG). The patient first presented with massive cervical lymphadenopathy at the age of one year. Biopsy revealed typical RDD; abnormally large CD68- and S-100 protein-positive histiocytes with occasional emperipolesis filled up the sinuses. Two years later, he developed polyuria and polydypsia. Skull film demonstrated osteolytic lesions at the occiput and left parietal region. Enlargement of the pituitary stalk was found on the magnetic resonance imaging. Despite the clinical impression of Langerhans cell histiocytosis, biopsy of the occipital lesion disclosed numerous large histiocytes with foamy cytoplasm. Several Touton giant cells with wreath-like arrangement of the nuclei were also observed. The abnormal cells expressed CD68 and factor XIIIa, but were non-reactive with S-100 protein and CD1a. Biopsy of the pituitary stalk was not performed According to the authors' literature search, this represents the first report of RDD and JXG affecting the same person.
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PMID:Rosai-Dorfman disease and juvenile xanthogranuloma in a Thai boy: report of a case. 1658 88

We present the case of a 10-week-old girl who had erythematous papules with a yellowish hue from birth with diagnosis of Langerhans cell histiocytosis, that was accompanied by a lytic lesion in the skull and hepatic involvement. After several months of treatment with prednisone and vinblastine with skin and systemic improvement, several rounded erythematous papules with a yellowish hue appeared in the right cheek. The biopsy showed a histiocytic infiltrate with positivity for CD68 and negative staining for S100 and CD1a, with a final diagnosis of juvenile xanthogranuloma. This association has been previously described in the literature in few cases. Although several hypotheses have been suggested, the causal relationship between both entities has still not been demonstrated.
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PMID:[Juvenile multiple xanthogranuloma in a patient with Langerhans cell histiocytosis]. 1717 66

We report a case of juvenile xanthogranuloma limited to involvement of the bone marrow in a 6-week-old male infant. Evaluation of the bone marrow was a part of the workup for peripheral blood cytopenia. Examination showed hypercellular marrow with paratrabecular clusters of lipidized histiocytes positive for CD68, CD4, and factor XIII(a) and negative for S100 and CD1a. Clinical and radiological workup showed no associated skin lesions or osseous or visceral involvement. The patient was started on chemotherapy with clinical improvement and gradual decreased bone marrow involvement. The child is alive and well at 16 months of age. This case represents, to the best of our knowledge, the 1st documented case of juvenile xanthogranuloma with isolated bone marrow involvement sparing skin and viscera.
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PMID:Isolated juvenile xanthogranuloma in the bone marrow: report of a case and review of the literature. 1737 89

Systemic juvenile xanthogranuloma is a rare disease in children. A 10-year-old boy who showed renal, pulmonary, and liver involvement is reported. He had pulmonary invasion, renal mass, and nodular liver lesions but no bone involvement. The diagnosis was confirmed by renal biopsy, which revealed foamy, lipid-laden macrophages with positive CD68, but negative CD1a and S-100. The patient was treated with pulse high-dose methylprednisolone (10 mg/kg/d for 3 d for 6 courses). On 1-year follow-up period after 6 courses therapy was resulted in remarkable regression in renal and liver lesions.
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PMID:Successful therapy of systemic xanthogranuloma in a child. 1755 9


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