Constrictive bronchiolitis obliterans and paraneoplastic pemphigus.
(17/1303)
Constrictive bronchiolitis obliterans is rare, and the pathogenesis of the disease often remains unknown. This study reports on the case of a 38 yr-old female with constrictive bronchiolitis obliterans and paraneoplastic pemphigus associated with malignant lymphoma. The patient developed progressive obstructive lung disease. The chest radiograph showed almost normal lungs. Paraneoplastic pemphigus is a newly described syndrome in which patients have autoantibodies binding to some epithelia, including in the respiratory tract. The disease develops in association with non-Hodgkin's lymphomas or other malignant neoplasms. The case presented here suggests that constrictive bronchiolitis obliterans associated with paraneoplastic pemphigus may be one of the facets of autoimmune responses in this context. (+info)
Cooperation of Myb and Myc proteins in T cell lymphomagenesis.
(18/1303)
The v-Myb oncogene causes late onset T cell lymphomas when expressed in the T cell lineage of transgenic mice. In order to define the cellular mutations cooperating with s-Myb to cause lymphomas, we have infected v-Myb transgenic mice with Moloney murine leukemia virus (M-MuLV). Tumor formation is significantly accelerated from a mean age of onset of 60 weeks in uninfected vMyb transgenics to 13 weeks in infected vMyb transgenics. We studied the loci into which the M-MuLV had inserted, and found that in 73% of animals, either the c-myc or the N-myc genes had been disrupted and deregulated. Therefore, v-myb and c-myb can cooperate to induce T cell lymphomas. (+info)
Mucosal intra-epithelial lymphocytes in enteropathy-associated T-cell lymphoma, ulcerative jejunitis, and refractory celiac disease constitute a neoplastic population.
(19/1303)
Loss of response to a gluten-free diet (refractory sprue) and ulcerative jejunitis are complications of celiac disease that may progress to enteropathy-associated T-cell lymphoma (EATL). Both conditions are characterized by the presence of a nonlymphomatous monoclonal T-cell population in the enteropathic mucosa. In EATL, a similar monoclonal population that shows clonal identity with the lymphoma itself is also present in the enteropathic mucosa. In this study we show that in all three circumstances the monoclonal T-cell population is constituted by cytologically normal, noninvasive intraepithelial T lymphocytes that share an identical aberrant immunophenotype with EATL. Patients with refractory sprue and/or ulcerative jejunitis are, therefore, suffering from a neoplastic T-cell disorder for which hematological treatment strategies need to be devised. (+info)
Immunogenicity of apoptotic cells in vivo: role of antigen load, antigen-presenting cells, and cytokines.
(20/1303)
Apoptosis allows the clearance of unwanted cells from living tissues without causing inflammation. Processing of phagocytosed apoptotic cells yields Ags that access the cytosol and the MHC class I pathway of engulfing cells and are recognized by Ag-specific CTL. We show here that injection of apoptotic RMA cells, a syngeneic T cell lymphoma, into C57BL/6 mice results in priming of a functional and long-lasting tumor-specific immune response. Cross-priming of CTLs by apoptotic cells requires CD4+ T cell help. Apoptotic cells, however, are at least 20-fold less immunogenic than nonreplicating live cells. Immunogenicity of apoptotic cells is proportional to the number of cells injected, correlates with the serum concentration of IL-10 and IL-1beta cytokines, and is enhanced in IL-10 knockout mice. Moreover, immunization with dendritic cells (DCs), but not macrophages (Mphi), pulsed with apoptotic cells primes tumor-specific CTLs and confers protection against a tumor challenge. Our findings demonstrate that tumor cells undergoing apoptosis are, though scarcely, immunogenic in vivo, outline the different roles of Mphi and DCs in the physiologic clearance of unwanted cells, and have implications in designing immunomodulating vaccines. (+info)
Primary central nervous system T-cell lymphoma. Case report.
(21/1303)
A 46-year-old male presented with a rare primary non-Hodgkin's lymphoma of the central nervous system of T-cell lineage, localized primarily in the right parietal region. There was no evidence of acquired immunodeficiency syndrome. Biopsy of the tumor allowed immunohistochemical confirmation of the diagnosis. Irradiation and chemotherapy were given, and the patient has remained well for 24 months. The clinical manifestations, management, and outcome of T-cell lymphoma are very similar to those of B-cell lymphoma. (+info)
Management of non-Hodgkin's lymphomas.
(22/1303)
The non-Hodgkin's lymphomas (NHL) are a heterogeneous group of disorders characterised by malignant proliferation of lymphoid cells. The cellular origin is relatively well established with subtypes corresponding to the various stages of lymphocyte differentiation. The term encompasses a hotchpotch of conditions with very different morphological appearance, behaviour and clinical outcome. NHL comprise 2.4% of all cancers, with incidence increasing with age. The commonest presentation is with progressive lymphadenopathy, though extranodal manifestations are present in a significant proportion. The clinical behaviour ranges from a benign, indolent course to rapidly progressive disease; prognosis varies from weeks to many years. Treatment is correspondingly diverse, from 'watchful waiting' to high-dose chemotherapy with bone marrow stem cell transplantation. Cure is possible in an increasing number of patients and much interest currently lies in identifying patients with high-risk disease necessitating the use of intensive treatment regimens. (+info)
T-cell lymphoma showing a non-enhancing diffuse white matter lesion with marked brain atrophy.
(23/1303)
We report an autopsy case of T-cell lymphoma with diffuse white matter infiltration. Cranial magnetic resonance (MR) images showed atrophy of the brain with a diffuse, non-enhancing, T2-high signal intensity lesion in the cerebral white matter. Intra-axial infiltration of T-cell lymphoma should be considered a differential diagnosis in patients with these MRI findings. (+info)
Successful treatment of advanced natural killer cell lymphoma with high-dose chemotherapy and syngeneic peripheral blood stem cell transplantation.
(24/1303)
CD56+ angiocentric lymphoma has currently been recognized as a distinct clinical entity which is the prototype of the putative NK cell lymphomas. A 16-year-old Japanese girl with advanced CD56+ angiocentric lymphoma received high-dose chemotherapy supported with syngeneic peripheral blood stem cell transplantation (PBSCT). Prior to syngeneic PBSCT, she received six cycles of conventional chemotherapy before transplantation, resulting in a partial response. PBSC were mobilized with granulocyte colony-stimulating factor (G-CSF) and collected from her identical twin. High-dose cyclophosphamide, MCNU, etoposide, and carboplatin were used for pretransplant conditioning. Syngeneic PBSCT was well tolerated. She achieved complete remission and is now surviving in continuous complete remission for more than 30 months after syngeneic PBSCT. Thus, marrow-ablative chemotherapy facilitated by autologous or allogeneic PBSCT should be considered as part of the primary therapy for poor prognosis NK cell lymphomas. (+info)