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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dendritic cells (DCs), the mononuclear cells that initiate immune response, and osteoclasts, the multinucleated bone-resorbing cells, are derived from monocyte/macrophage precursor cells. Granulocyte-macrophage colony-stimulating factor and macrophage colony-stimulating factor (M-CSF) reciprocally regulate the differentiation of both lineages in mice. Using human monocyte-derived DCs generated in vitro, we show that immature DCs transdifferentiate into functional osteoclasts (OCs) in the presence of M-CSF and receptor activator of nuclear factor-kappaB ligand (RANKL). Transdifferentiation operates through fusion of intermediate adherent bipolar fusiform mononuclear cells expressing CD14, CD1a, and RANKL and able to induce RANKL(+) T-cell proliferation. Surprisingly, DC fusion in vitro is faster and more efficient than monocyte fusion to form multinucleated giant cells. The transdifferentiation process reported here supports the existence of a high cellular plasticity within differentiated myeloid phagocytes. Importantly, this process is greatly enhanced by rheumatoid arthritis synovial fluid and involves proinflammatory cytokines such as interleukin 1 or tumor necrosis factor alpha, as well as components of the extracellular matrix such as hyaluronic acid. Our data therefore suggest that DC-derived OCs may be directly involved in the osteolytic lesions observed in human inflammatory bone diseases such as rheumatoid arthritis or in particular forms of Langerhans cell histiocytosis, characterized by accumulation of immature skin DCs and chronic lytic bone lesions.
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PMID:Immature dendritic cell transdifferentiation into osteoclasts: a novel pathway sustained by the rheumatoid arthritis microenvironment. 1530 76

We make a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We report a case of Erdheim-Chester disease and review 60 cases from the literature. These cases are consider to have Erdheim-Chester disease when they have either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration with distinctive immunohistochemical phenotype of the non-Langerhans cell histiocytes with positive staining for CD68 and negative staining for S-100 protein and CD1a. Our patient undergoes chemiotherapy according to the LCH-II stratification and therapy plan (Vinblastine, Etoposide and Prednisone) and thereafter receives Carboplatin and Etoposide, and Somatostatin. She is alive and clinically well 33 months after onset of symptoms and the lesions don't appear to progress at imaging examinations. In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, the characteristics radiological pattern of Erdheim-Chester disease together the immunohistochemical phenotype of hystiocytic infiltration supports the theory that Erdheim-Chester disease is a unique disease entity distinct.
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PMID:[Erdheim-Chester disease: a non-Langerhans cell histiocytosis. A clinical-case and review of the literature]. 1534 69

A 57-year-old man who was a heavy smoker was admitted to our hospital for further evaluation of abnormal shadows on a chest X-ray film. Chest radiography and a computed tomography (CT) scan revealed nodular lesions and multiple thin-walled cysts in both lungs. Histopathological examination of one of these cystic lesions showed that the predominant cellular population was Langerhans cells, with the cytoplasm testing positive for S-100 protein and the cell membrane showing a positive reaction for CD1a. The pathological diagnosis was pulmonary Langerhans cell histiocytosis (LCH). A lingual carcinoma that had been detected simultaneously was treated with neoadjuvant therapy and the patient was advised to stop smoking. However, only limited improvement was seen on follow-up chest CT. In view of this, a radical resection of the lingual carcinoma was performed. There was a subsequent dramatic improvement in the pulmonary LCH. Langerhans cells may play a role in the immune response to tumors. In this patient, we suggest the possibility that both the habitual smoking and the lingual carcinoma may have contributed to the development of pulmonary LCH.
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PMID:Pulmonary Langerhans cell histiocytosis associated with lingual carcinoma. 1546 72

A cutaneous lesion as sole manifestation of adult Langerhans cell histiocytosis is rare. We report a 65-year old female who presented with vulvar ulcers. Histological examination of the ulcer showed diffuse proliferation of histiocytic cells with large bright cytoplasm that were positively stained for anti-S-100 protein and anti-CD1a antibodies by immunohistochemistry. Electron microscopy demonstrated Birbeck granules in the cytoplasm of the cells. Since there was no other organ involvement, the patient was treated by complete surgical excision without recurrence or other-organ involvement in the 1-year follow-up period.
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PMID:Localised vulva Langerhans cell histiocytosis. 1556 6

We report a case of pulmonary Langerhans' cell histiocytosis with liver involvement in an adult. An asymptomatic 51-year-old man who had, until his referral to our hospital, been a smoker was suspected on examination to have multiple metastases to the lungs and liver. Chest CT showed multiple micronodules, located predominantly in the upper and middle lung fields. Many of these nodules were cavitary. Abdominal US showed multiple, well-defined, round and oval nodules of decreased echogenicity within the liver. Hepatic nodules were enhanced rapidly by contrast-enhanced CT. Comparatively large nodules were demonstrated as well-circumscribed hypoattenuating lesions with ring enhancement. Since further work-up did not demonstrate any evidence of malignancy, open lung biopsy was performed. The results of open lung biopsy were consistent with Langerhans' cell histiocytosis. The infiltrates consisted of variable numbers of Langerhans' cells with folded nuclei and eosinophils. Immunohistochemically, the histiocytes were positive for S-100 protein and CD1a, and negative for CD68. The nodules regressed within 5 months after smoking cessation, and the patient became free of disease 10 months after the initial presentation.
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PMID:[A case of pulmonary Langerhans' cell histiocytosis with liver involvement]. 1556 9

Juvenile xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytosis. We investigated 148 biopsy specimens from 129 patients collected in the Kiel Pediatric Tumor Registry (KPTR) between 1965 and 2001. The clinical, histologic, and immunohistochemical characteristics of JXG were evaluated to gain more and deeper insights into the morphology and clinical behavior of JXG. Conventionally stained lesions were classified into the following morphologic subtypes: early JXG (EJXG), classic JXG (CJXG), transitional JXG (TJXG), or combined lesions with more than one basic pattern (combined JXG). Immunohistochemistry included antibodies against macrophages (Ki-M1P), S-100 protein, CD1a, and factor XIIIa (FXIIIa). Clinical data were obtained by means of a standardized questionnaire. The relative incidence of JXG in the KPTR is 0.52%. The male/female ratio was 1.4:1. The mean age was 22.4 months (median, 5 months; range, 0-244 months). A total of 34.5% of the cases of JXG were congenital, and 71.0% of the lesions were diagnosed within the first year of life. Most cases of cutaneous JXG were solitary (81.0%). Five cases (3.9%) presented with visceral (systemic) involvement. Histologically, CJXG was most frequent (47.2%), followed by EJXG (27.1%) and TJXG (16.0%). A total of 9.7% of the lesions represented combined JXG. Histiocytes, including giant cells, were positive for Ki-M1P (100%) and in most cases for FXIIIa (99%). The CD1a and S-100 protein reactions were generally negative. Clinical and follow-up data showed a generally favorable prognosis with a low relapse rate (7.0%) and even complete involution after incomplete resection. Only 1 of 5 patients with widespread congenital systemic disease died after 34 days. JXG is an uncommon, mostly cutaneous, and prognostically favorable histiocytic tumor of infancy. Simple tumor excision is the therapy for choice except in the very rare systemic JXG, in which multimodal chemotherapy is indicated.
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PMID:Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry. 1600 12

We describe a new case of isolated Langerhans cell histiocytosis (LCH) of the hypothalamus. A 53-year-old female patient presented with polydipsia, headache, anorexia, and fatigue. Neurological imaging revealed a mass projecting from the hypothalamus into the third ventricle. Gross total removal of the tumor was performed. Light microscopy showed LCH, and immunohistochemical studies revealed S-100 and CD1a immunoreactivity in the Langerhans cells. Although the most common CNS site for LCH is the hypothalamus, isolated hypothalamic LCH, confirmed by biopsy, is very rare. The residual mass appeared to remit spontaneously 3.5 years after surgery, with regrowth 3 years later.
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PMID:A case of isolated langerhans cell histiocytosis of the hypothalamus with remission and regrowth after surgery. 1570 Aug 40

CNS involvement in Langerhans cell histiocytosis (LCH) is a rare but potentially devastating disorder. Different types of involvement have been described by MRI. CNS changes can have space-occupying or degenerative character. Little is known about the underlying neuropathology and pathophysiology. In our study we reviewed brain samples from 12 patients with LCH. The neuropathology findings were correlated with the MR morphology and the clinical presentation. By neuropathology, three types of lesions were distinguished. (i) Circumscribed granulomas within the brain's connective tissue space corresponded to tumorous lesions in the meninges or choroid plexus on MRI. They showed a composition similar to Langerhans granulomas in peripheral organs, with variable presence of CD1a-reactive cells and pronounced CD8-positive (+) T-cell infiltration. (ii) Granulomas occur within the brain's connective tissue spaces with partial infiltration of the surrounding CNS parenchyma by CD1a-reactive histiocytes. This was associated with profound T-cell-dominated inflammation and severe neurodegeneration, characterized by a nearly complete loss of neurons and axons, and gliosis. (iii) Neurodegenerative lesions lacking infiltration of CD1a+ cells, mainly affecting the cerebellum and brainstem, exhibited a profound inflammatory process dominated by CD8-reactive lymphocytes, associated with tissue degeneration, microglial activation and gliosis. Patients with such lesions showed different stages of neurological deterioration. This study indicates that neurodegeneration in LCH occurs on the background of a T-cell-dominated inflammatory process and is characterized by neuronal and axonal destruction with secondary demyelination, resembling paraneoplastic encephalitis.
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PMID:Neuropathology of CNS disease in Langerhans cell histiocytosis. 1570 14

Langerhans cell histiocytosis (LCH) is a disease that can involve one or multiple organ systems characterized by an accumulation of CD1a(+) Langerhans-like cells as well as several other myeloid cell types. The precise origin and role of one of these populations, the multinucleated giant cell (MGC), in this disease remains unknown. This work shows that in three different lesional tissues, bone, skin, and lymph node, the MGCs expressed the characteristic osteoclast markers, tartrate-resistant acid phosphatase and vitronectin receptor, as well as the enzymes cathepsin K and matrix metalloproteinase-9. Although, in bone lesions, the osteoclast-like MGCs were only CD68(+), in the nonostotic sites, they coexpressed CD1a. The presence of osteoclast-like MGCs may be explained by the production of osteoclast-inducing cytokines such as receptor activator of nuclear factor kappaB ligand and macrophage colony-stimulating factor by both the CD1a(+) LCH cells and T cells in these lesions. As osteoclast-derived enzymes play a major role in tissue destruction, the osteoclast-like nature of MGCs in all LCH lesions makes them a potential target for the treatment of this disease.
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PMID:Presence of osteoclast-like multinucleated giant cells in the bone and nonostotic lesions of Langerhans cell histiocytosis. 1575 4

Langerhans cell histiocytosis (LCH) is a disorder characterized by neoplastic proliferation of Langerhans cells that rarely involves the skin in adults. A 74-year-old woman presented with a fourteen year history of eosinophilic granuloma and bone involvement caused by LCH. She had received three combination therapy courses of curettage and radiation since 1987 and had remained free of LCH signs for seven years, after which she started to notice brown nodules on her left leg. Biopsy specimens taken from the lesions showed massive proliferations of large histiocytic cells. Immunoperoxidase stainings for CD1a and S-100 protein were positive. Electron microscopy identified Birbeck granules in the cytoplasm of the atypical Langerhans cells. Treatment with oral prednisolone alone has resulted in the patient remaining in complete remission for 12 months.
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PMID:Langerhans cell histiocytosis involving the skin of an elderly woman: a satisfactory remission with oral prednisolone alone. 1580 Dec 69


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