Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumonia (asymptomatic). The relationship of the vesicles and pneumonia to the histiocytic disorder is not clear. Ultrastructurally, worm-like (comma-shaped) particles, dense bodies and Birbeck granules were not found. Histiocytes were Leu-6 negative, and S100 (partly), Leu M3 and HLA-DR positive. Positive reactions were also obtained with anti-lysozyme and non-specific esterase. Several aspects of this case and of others described previously are discussed.
 ...
PMID:Congenital self-healing non-Langerhans cell histiocytosis. 305 80

The authors describe a 63-year-old woman who developed a histologically distinctive malignant cutaneous neoplasm composed of large pleomorphic cells with abundant cytoplasm and multilobate, often clefted nuclei that occasionally contained small nucleoli. This neoplastic cell population metastasized to a regional lymph node already involved by a B-cell derived chronic lymphocytic leukemia expressing surface IgMk, BA-1, and OKT1. The large metastatic tumor cells lacked surface immunoglobulin, B-lymphocyte associated antigen BA-1, T-lymphocyte associated antigens OKT1 and OKT3, and the monocyte/macrophage markers lysozyme and alpha 1-antichymotrypsin. These tumor cells expressed HLA-DR antigens, adenosine triphosphatase (ATPase), OKT6, and contained S-100 protein, i.e., they expressed the phenotype peculiar to epidermal Langerhans cells. The typical clinical and histologic features of Histiocytosis X were absent. Thus, this case appears to represent a distinctive cutaneous neoplasm composed entirely of malignant cells of dendritic cell origin which, by immunophenotypic and histochemical analysis, appear to be related to epidermal Langerhans cells.
 ...
PMID:A distinctive cutaneous malignant neoplasm expressing the Langerhans cell phenotype. Synchronous occurrence with B-chronic lymphocytic leukemia. 388 25

We studied a unique case of a localized non-Hodgkin's lymphoma of the pleomorphic large-cell type arising in the small intestine to determine its phenotype. Immunohistochemical staining for S100 protein, lysozyme, alpha 1-antitrypsin, and Leu-M1 was performed. Many lymphoma cells were positive for S100 protein and were negative with the other antibodies. These findings indicate a probable dendritic cell origin for this lymphoma similar to that seen in histiocytosis X and some cases of malignant histiocytosis, but apparently quite distinct from the S100 protein-negative, lysozyme-positive, alpha 1-antitrypsin-positive cells seen within the mononuclear phagocytic system.
 ...
PMID:Dendritic cell phenotype in localized malignant histiocytosis of the small intestine. 389 79

Histiocytosis X, multicentric reticulohistiocytosis, juvenile xanthogranuloma, the "fibrous" type of dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma are all characterized by dermal and/or subcutaneous infiltrates composed at least partially of cells having morphologic features suggestive of histiocytes. Paraffin-embedded tissues representing these conditions were stained for lysozyme (muramidase) with a peroxidase-antiperoxidase technic. The cells of juvenile xanthogranuloma were rich in lysozyme. Some of the cells of histiocytosis X showed a positive pattern, and the cells of the other three conditions were essentially negative. This study confirmed the histiocytic nature of juvenile xanthogranuloma and multicentric reticulohistiocytosis, supported the interpretation that there is a histiocytic component in the lesions of histiocytosis X, and cast some doubt on the alleged histiocytic nature of "fibrous" dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma.
 ...
PMID:Immunohistochemical identification of lysozyme in cutaneous lesions of alleged histiocytic nature. 625 20

The cellular nature of the proliferating histiocytes in 6 cases of histiocytosis X was studied immunohistochemically and ultrastructurally. Immunohistochemically, S-100 protein was detected both in the cytoplasm and the nuclei of histiocytosis X cells as well as Langerhans cells in normal oral epithelium. These cells were always negative for lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin and immunoglobulins. S-100 protein was not detected in lysozyme-positive histiocytes and multinucleated giant cells often showed the signs of phagocytosis. Thus, S-100 protein appears to be a useful immunohistochemical marker for histiocytosis X cells. Ultrastructurally, Birbeck granules noticed in histiocytosis X cells were never seen in the phagocytic histiocytes with numerous lysosomes and phagosomes. These results emphasized the heterogeneous nature of the proliferating histiocytes involved in the lesions. Since histiocytosis X cells share characteristics, not only ultrastructurally but also immunohistochemically, with Langerhans cells, the hypothesis that histiocytosis X may be fundamentally an abnormal proliferation of Langerhans cells has been further supported.
 ...
PMID:Immunohistochemical and ultrastructural analysis of the proliferating cells in histiocytosis X. 636 33

Using the peroxidase antiperoxidase (PAP) method, lysozyme (LZM) was shown to exist in normal, reactive and neoplastic cells belonging to the mononuclear phagocyte system (MPS), but was not detected in histiocytosis X cells. Immunostaining for cytoplasmic LZM by the PAP method is useful for identification of mononuclear phagocytes and for diagnosis of the diseases in which these cells participate.
 ...
PMID:Immunohistochemical demonstration of lysozyme in normal, reactive and neoplastic cells of the mononuclear phagocyte system. 637 69

In this study the antigenic profile of Hodgkin (H) and Sternberg-Reed (SR) cells from cases of Hodgkin's disease was analysed using a large panel of monoclonal and polyclonal antibodies reactive with cells of lymphoid and haemotopoietic origin. The aim of this investigation was, firstly, to throw light on the origin of H and SR cells and, secondly, to determine whether there is any evidence to support recent suggestions that H and SR cells differ antigenically between different histological categories of Hodgkin's disease. Frozen sections (from 24 cases) and paraffin sections (83 cases) were stained by immunoenzymatic methods and the results compared with those obtained from staining a wide variety of reactive and neoplastic tissue samples (including examples of tuberculosis, sarcoidosis, malignant histiocytosis, histiocytosis X, osteomyelosclerosis and non-Hodgkin's lymphoma). The results revealed that H and SR cells of all types of Hodgkin's disease consistently lack markers found on null cells, B cells, T cells, cells of monocyte/macrophage series, interdigitating reticulum cells, dendritic reticulum cells and erythropoietic and thrombopoietic cells. However, H and SR cells constantly expressed an antigen detectable with the recently produced monoclonal antibody Ki-I. The vast majority of typical and lacunar type H and SR cells contained the granulocyte-related antigens detected by monoclonal antibodies TU5, TU6, TU9 and 3C4, whereas other more or less specific granulopoietic cell markers (such as peroxidase, chloroacetate esterade, lysozyme, cationic leukocyte antigen and OKMI) were consistently absent. H and SR cells in cases of nodular paragranuloma (nodular type of Hodgkin's disease with lymphocyte predominance) were not monotypic in light chain type (as has been previously reported), but rather contained chi and lambda chains within the same cells, as do typical and lacunar type H and SR cells. Immunostaining of normal and hyperplastic lymphoid tissue with the Ki-I antibody led to the detection of a new, as yet unidentified, small-cell population of unknown origin and function, which is present between, around, and within cortical follicles. It is concluded from these findings that H and SR cells constitute a unique cell type that differs in many properties from all other known cell types. Furthermore, H and SR cells of the various histological types of Hodgkin's disease are more closely related than previously believed. It is suggested that the hitherto unknown cell population detected with the monoclonal antibody Ki-I in normal lymphoid tissue is the normal equivalent of H and SR cells.
 ...
PMID:Identification of Hodgkin and Sternberg-reed cells as a unique cell type derived from a newly-detected small-cell population. 675 30

A clinicopathologic study of histiocytosis X in lymph nodes disclosed a special variant: primary eosinophilic granuloma of lymph nodes. This variant involves one or more lymph nodes, but does not infiltrate any other organs. Histologically, the infiltration of lymph nodes by histiocytosis X cells and eosinophils is similar to that seen in disseminated or metastatic histiocytosis X. Most cases of eosinophilic granuloma of lymph nodes are recognizable as primary, however, by the heavy infiltration of the surrounding tissue. The predominant proliferating cells are histiocytosis X cells ('Langerhans cells'), which contain Birbeck granules on electron microscopy and are lysozyme-negative. The disease was found in 30 patients among a total of 64 cases of histiocytosis X collected at the Lymph Node Registry in Kiel. Primary eosinophilic granuloma of lymph nodes occurs predominantly in children and young adults and shows a slight preponderance of males. Clinically, the patients present with mostly afebrile and sometimes painful lymphadenopathy, which is more often solitary (in the cervical or inguinal region) than widespread. The erythrocyte sedimentation rate and/or serum alpha 2-globulin level are elevated in many patients. There may also be an increase in the number of leucocytes, especially eosinophils, in the blood. The prognosis is favourable: the lymphadenopathy disappeared spontaneously in most patients and only one patient developed two recurrences. Thus, primary eosophilic granuloma of lymph nodes is interpreted as a benign lesion. It might be a special reaction of the T cell system.
 ...
PMID:Eosinophilic granuloma of lymph nodes--a variant of histiocytosis X. 696 10

The immunophenotype and proliferation fraction have been investigated in 26 cases of Langerhans' cell histiocytosis (LCH). In all cases LCH cells were positive for S-100 protein, CD1a, or both. In most cases LCH cells expressed the macrophage-associated marker CD68 and in two cases they contained lysozyme. Expression of both cytoplasmic CD2 and CD3 was observed in cryostat sections. An unexpected finding was the presence of placental alkaline phosphatase in LCH cells. Langerhans' cells in normal skin were negative for both CD2 and CD3, but a proportion contained placental alkaline phosphatase. In four cases of Rosai-Dorfman disease the histiocytic cells, which share certain immunophenotypic properties with Langerhans' cells, also were positive for placental alkaline phosphatase. A significant proportion of LCH cells stained positively with the antibody to proliferating cell nuclear antigen and also with the proliferation marker Ki-S1. A good correlation between the percentage of Ki-67-positive and proliferating cell nuclear antigen- and Ki-S1-positive cells, respectively, was observed. Thus, in comparison with their putative precursors, LCH cells have an aberrant phenotype and are proliferating locally. This might suggest that LCH is a neoplastic rather than a reactive process.
 ...
PMID:Langerhans' cell histiocytosis (histiocytosis X): immunophenotype and growth fraction. 769 Jul 35

Although several attempts at the immunohistochemical characterization of histiocytosis have recently been made there is only one paper which reports a case of cerebral Langerhans cell histiocytosis (LCH) diagnosed by biopsy. This paper presents a bioptically diagnosed case of juvenile histiocytosis. The panel of antibodies used was as follows: anti-S-100, 2 different antibodies to anti-interleukin 2, anti-lysozyme, anti-LEU M1, anti-MAC 387, anti-major histocompatibility complex II and anti-GFAP. Microglia markers--Griffonia simplicifolia and RCA 1 lectins were also utilized. The proliferating cells produced a positive response to S-100, lysozyme and a partially positive response to HLA DR, but responded negatively to MAC 387, LEU M1, lectins, IL2R and GFAP. Our results were compared and analyzed in the light of those obtained by other authors.
 ...
PMID:Immunohistological study of a case of cerebral Langerhans cell histiocytosis in brain biopsy. 772 76


<< Previous 1 2 3 4 Next >>