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Query: UMLS:C0019621 (
Langerhans cell histiocytosis
)
3,250
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The expression of macrophage antigens KP1, Mac, lysozyme, and alpha-1-antichymotrypsin was investigated on routine paraffin sections from 17 cases of
Langerhans' cell histiocytosis
(
LCH
). All the major clinical forms were represented, including single lesions and monosystemic and multisystemic disease. In all the cases, a variable fraction (3-35%) of
LCH
cells was immunoreactive with KP1 and anti-Mac; the staining pattern was quite typical because the immunoreaction product was often confined to the perinuclear space and the Golgi area.
LCH
cells containing lysozyme and AACT were detected less frequently; however, in positive cases the percentage of
LCH
cells immunoreactive for lysozyme and AACT was in the same range as that of KP1-positive cells. On immunostained cytosmears (one case), about 10% of the CD1a-positive cell population was reactive for the macrophage antigens CD14 and
PAM-1
. No association was noted between the number of KP1-positive cells and the clinical form and/or anatomic site of the lesion. Phagocytic macrophages were significantly and diffusely immunoreactive with KP1 and anti-Mac and for AACT and lysozyme. Multinucleated giant cells with irregular nuclei were frequently observed; these cells were rarely S-100 positive, were consistently stained by KP1 and AACT, and were occasionally anti-Mac positive. The authors' findings suggest that antimacrophage monoclonals, in conjunction with S-100 protein, may represent a useful tool to establish the diagnosis of
LCH
in paraffin-embedded material.
...
PMID:Expression of macrophage-associated antigens in tissues involved by Langerhans' cell histiocytosis (histiocytosis X). 278 88
The morphological, ultrastructural and immunophenotypic properties of
Histiocytosis-X
(H-X) cells were investigated in a lymph node involved by Letterer-Siwe (L-S) disease. H-X cells were T6+ (CD1a), S-100+, T4+ (CD4) and HLA-DR+; in addition they were consistently T11+ (CD2) and were stained by antibodies directed against receptors for transferrin (T9), C3bi (OKM-1/CD11b), IgG-Fc (Leu-11/CD16) and Interleukin-2 (IL-2R/CD25). On immunostained cytosmears, T6+ cells were highly polymorphic and a prominent fraction (45%) showed immature morphology, characterized by lymphoid appearance. Cells expressing macrophage markers (ANAE, AACT, Leu-M3/CD14,
PAM-1
) were 10-fold fewer than T6+ cells and did not show a lymphoid morphology. At TEM level, H-X cells were characterized by poor content of LC granules and by the presence of myelin-like laminated bodies and of lysosome-like dense bodies. The immunophenotypic properties of H-X cells were compared to those of epidermal Langerhans cells (LCs) and of LCs present in lymph nodes of three cases of dermatophatic lymphadenitis. Epidermal LCs were T6+/HLA-DR+, and sometimes faintly T4+. Lymph node LCs were T6+, S-100+, T4+, HLA-DR+, and showed the same variety of surface receptors detected in H-X cells; furthermore, in a case with massive infiltration of the paracortex by T6+ cells, lymph node LCs were faintly T11+ and some of the T6+ cells had lymphoid aspect. Our findings suggest that the H-X cell population of L-S disease is not homogeneous, but is composed of discrete cell subsets with distinctive antigenic and morphological traits closely resembling those of cells of LC lineage at different maturational stages.
...
PMID:Letterer-Siwe disease: immunohistochemical evidence for a proliferative disorder involving immature cells of Langerhans lineage. 313 61
