1/6. Lethal midline granuloma (peripheral T-cell lymphoma) after lymphomatoid papulosis.A Japanese woman with an 8-year history of lymphomatoid papulosis (LP) had lethal midline granuloma (LMG) develop at the age of 51 years. There were histologic similarities between LP and LMG seen in this patient. Surface phenotypic studies on nasal and cutaneous lesions demonstrated a population of T-cells expressing CD2, CD4, CD25, CD30, and histocompatibility antigen-DR (HLA-DR). Genotypic analyses of nasal and skin biopsy specimens disclosed a clonal rearrangement of the beta T-cell receptor gene with the same rearrangement pattern. These data indicate that this patient had LMG characterized by clonal peripheral T-cell lymphoma, which probably resulted from progression of the LP.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/6. association of Epstein-Barr virus with polymorphic reticulosis.We report a case of polymorphic reticulosis (PR) associated with pharyngeal replication of Epstein-Barr virus (EBV). A 78-year-old man with necrotic inflammatory granulations in the nasal cavities and ulcerative tissue of the tonsils was found to have PR after an initial diagnosis of lethal midline granuloma and was found to have high antibody titers to EBV. EBV-determined nuclear antigen (EBNA) was demonstrated in pharyngeal biopsy specimens by the anti-complement immunofluorescent technique, following which dual immunofluorescence staining, EBNA and T-cell antigen analysis were performed, using a wide variety of monoclonal antibodies. All of the EBNA-positive lymphocytes in the pharyngeal biopsy specimens were found to have exclusively T-cell antigens. This case strongly suggests that some of the cases of PR related to T-cell lymphomas may be closely associated with active EBV infection.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
3/6. Lethal midline granuloma (polymorphic reticulosis) and lymphomatoid granulomatosis. Evidence for a monoclonal T-cell lymphoproliferative disorder.lymphomatoid granulomatosis (LG) and polymorphic reticulosis (PR), originally described as distinct entities, now are considered as a single disease process. Common histopathologic features include necrosis, vasculitis, and a granulomatous infiltrate. Such features have led to consider lymphomatoid granulomatosis as a systemic vasculitis; alternatively the possible emergence of an overt lymphoma has suggested that it could be a lymphoproliferative process. To investigate this later hypothesis, the authors analyzed the cellular infiltrate of tissue specimens from two patients with histologic features of LG. The analysis included the study of T-cell antigen expression and dna rearrangement of the beta T-cell receptor gene. In one patient, the T-cell phenotype of infiltrating cells was abnormal because of antigen loss. In both patients, the cells contained rearranged dna indicating the presence of a clonal T-cell proliferation. It is concluded that some cases of LG and PR, if not all, are related to a neoplastic T-cell lymphoproliferative disorder.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/6. Malignant histiocytosis (histiocytic sarcoma). A (the?) major cause of the 'midline granuloma syndrome'.Five out of eight consecutive cases with initial symptoms of a 'midline granuloma' were identified as malignant histiocytosis (histiocytic sarcoma) which within 5 months to 4 years led to generalization and death. The three remaining cases also fulfilled the morphological criteria of this type of neoplasia, though these patients are still alive 1/2 to 8 years after diagnosis, possibly as a result of local radiotherapy. The age of the individuals ranged from 18 to 71 years and there was a male preponderance of 7:1. The histiocytic nature of the atypical cells was primarily documented by intense activity of NaF-inhibitable non-specific esterase, of acid phosphatase and of beta-glucuronidase as demonstrated in cryostat sections of formaldehyde-saccharose-fixed fresh biopsy specimens and by the detection of alpha-1-antichymotrypsin, alpha-1-antitrypsin, and lysozyme antigens, in that order of constancy (immunohistochemical examination of formaldehyde-fixed paraffin sections, using the avidin-biotin-peroxidase complex method). There was among the reported cases a considerable heterogeneity with regard to these 'markers'. We conclude that malignant histiocytosis is a (the?) major cause of the 'midline granuloma syndrome'.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/6. Midfacial T cell lymphoma: characterization by monoclonal antibodies.Three cases of so-called lethal midline granuloma were studied immunologically, using monoclonal antibodies against T cell subsets, and electron microscopically. Immunofluorescence and immunoperoxidase studies of their surgical and autopsy specimens proved that the tumor cells showed positive stainings with anti-Leu 1 and anti-Leu 3a antibodies, and negative staining with anti-Leu 2a, anti-HLA-DR, and anti-immunoglobulin antibodies. These data might indicate that the tumor cells of the three cases had a similar surface antigen phenotype to that of peripheral helper-inducer T cells. The histopathological and ultrastructural examinations of the tumors showed general characteristics reported for T cell lymphomata derived from peripheral T cells.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/6. radiation therapy of midline granuloma.During a 15-year period, 10 patients with well-documented midline granuloma were treated with high-dose, deep local irradiation and followed for extended periods of time. Long-term remissions were achieved in 7 patients, with a mean ( /- SEM) survival postirradiation of 7.4 ( /- 1.4) years in the 6 patients still alive. True midline granuloma, which is a localized, destructive, inflammatory process of the upper airways, can be distinguished from Wegener's granulomatosis and neoplasms of the upper respiratory tract by several clinicopathologic criteria. The cause of midline granuloma is unknown, but it most likely represents an abnormal accelerated hypersensitivity reaction to an unknown antigen(s). Although serious complications of high-dose local irradiation to the upper airways can occur, the risk is warranted because of the high, long-term remission rate in this previously uniformly fatal disease.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |