Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A factor that augmented the phagocytosis of IgG-coated ox red blood cells by the human monocyte/macrophage line U937 was identified in cell culture supernatants from two of two patients with angiocentric peripheral T cell lymphomas, three of three patients with angiocentric immunoproliferative lesions that were not frankly malignant, and one of two patients with T lymphoblastic malignancies. The factor was not present in supernatants derived from 14 nonangiocentric peripheral T cell lymphomas of other histologic types nor in ten cases of B cell lymphoma and two cases of Hodgkin's disease. A similar factor was present in the supernatants of concanavalin A (Con A)-stimulated normal peripheral blood mononuclear cells and in the supernatants of IL-2-dependent T cell lines derived from normal peripheral blood. The factor had an apparent mol wt of greater than 50,000 daltons, was heat labile (100 degrees C for two minutes), and stable at pH 2.0. Its stimulation of phagocytosis was independent of any increase in number of Fc receptors. Thus, this factor is probably not gamma-interferon. This factor may play a pathogenetic role in the hemophagocytic syndromes associated with certain T cell malignancies and immunodeficient states.
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PMID:Lymphokine-induced phagocytosis in angiocentric immunoproliferative lesions (AIL) and malignant lymphoma arising in AIL. 387 65

We studied open-lung biopsies from 17 patients with pulmonary lymphomatoid granulomatosis (LYG) using paraffin-section immunostains and Epstein-Barr virus (EBV) RNA in situ hybridization to assess the phenotype of these unique tumors and to clarify the role of EBV infection. Histologically, all cases demonstrated the characteristic mixed mononuclear cell infiltrate of lymphomatoid granulomatosis with variable numbers of cytologically atypical large lymphoid cells in a background of small lymphocytes. Paraffin-section immunostains in all cases showed a predominance of T lymphocytes. A minor population of CD20-positive large B lymphocytes was identified in 11 cases; immunoglobulin light-chain restriction was demonstrated in four of these and immunoglobulin gene rearrangements in another case. Nuclear labelling for EBV RNA was detected in 10 of these 11 cases and was confined to the population of large B lymphocytes. Staining for CD20 was absent in the remaining six cases, as was nuclear labeling for EBV RNA. However, the large atypical lymphoid cells stained for T-cell-lineage-specific antibodies in three of these cases. We conclude that some cases of lymphomatoid granulomatosis are B-cell lymphoma associated with EBV infection, whereas others are of T-cell origin and are probably unrelated to EBV infection.
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PMID:Lymphomatoid granulomatosis. Evidence of immunophenotypic diversity and relationship to Epstein-Barr virus infection. 757 93

To elucidate the relationship between the clinical features and pathologic findings of primary pulmonary lymphoma, we reviewed 24 patients with this disease. The pulmonary lymphomas were divided into four groups: (1) B-cell lymphoma composed of small to medium-sized lymphoid cells (19 cases); (2) B-cell lymphoma composed of large lymphoid cells (three cases); (3) T-cell lymphoma (one case); (4) malignant lymphoma of lymphomatoid granulomatosis (LYG) type (one case). Radiographs of the first group revealed a predominance of infiltration associated with ill-defined tumor margins upon gross pathology, corresponding histologically to lymphangitic spread. Air bronchogram and pleural tail or abutment were additional radiographic features. Characteristics of the second group were a nodule or mass evident on radiographs and well-circumscribed tumor margins upon gross pathology. Lack of air bronchogram was an another radiographic feature in this group. Seventeen patients in these two groups underwent complete resection of the tumors and survived without recurrence, whereas four received chemotherapy after biopsy and survived with disease. These results indicate that primary pulmonary B-cell lymphoma is a low-grade malignancy and that complete resection is the only therapy which leads to cure. In a single patient with T-cell lymphoma, the radiographic and pathologic features of the tumor were indistinguishable from those in the first group, but the patient had an unfavorable prognosis. We consider that, from a prognostic viewpoint, it is important to determine the T- or B-immunophenotype of the tumor cells for diagnosis of primary pulmonary lymphoma. The only patient in this series with pulmonary lymphoma of LYG type showed distinctive clinicopathologic findings. We consider that this uncommon disease should be separated from other types of primary pulmonary lymphoma.
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PMID:Primary pulmonary lymphoma: relationship between clinical features and pathologic findings in 24 cases. The Japan National Chest Hospital Study Group for Lung Cancer. 766 90

Clinicopathologic features in 14 cases of lymph node-involved angiocentric immunoproliferative lesions (AILs) are reported. They were selected from 900 cases of lymphoproliferative disorders registered at the Department of Pathology, Fukuoka University. Four cases showed a histologic feature of AIL grade II (AIL-II) and 10 had angiocentric lymphoma (AIL-III). Immunohistologically, transformed B cells were mixed with a large number of small T cells in AIL-II. In AIL-III, there were five cases with B-cell lymphoma, and three had peripheral T-cell lymphoma with no expression of natural-killer (NK)-associated antigens. In the remaining two cases, lymphoma cells expressed both T-cell- and NK-associated antigens. These findings indicate that lymph node-involved AILs are rarely occurring (1.6%) and phenotypically different from sinonasal and cutaneous AILs. Furthermore, NK-associated antigen-positive AILs were found to rarely involve the lymph node. For Epstein-Barr virus (EBV) infection, seven cases of AILs showed many atypical lymphocytes that were positive for EBV-encoded RNA (EBER-1) by using the in situ hybridization analysis. Among them, six cases had latent membrane protein (LMP) positive and EBV nuclear antigen 2 (EBNA-2) negative atypical lymphocytes. The pattern of latent EBV infection was similar to that of Hodgkin's disease, but differed from those of sinonasal T-cell lymphoma and other subtypes of non-Hodgkin's lymphoma. Clinically, 12 patients, including all 4 AIL-II, died within 22 months of the onset of the disease, despite intensive therapy, suggesting that lymph node-involved AILs have a poor prognosis.
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PMID:Angiocentric immunoproliferative lesions of the lymph node. 870 36

There are three distinct clinico-anatomical entities today covered by the definition of a primary clonal pulmonary lymphoid proliferation. These are pulmonary lymphomas of B cell phenotype, of low grade malignancy, B cell lymphomas of high grade malignancy and finally lymphomatoid granulomatosis whose clonal characteristic is sometimes difficult to confirm. This general review aims to specify the pathophysiological, diagnostic, prognostic and therapeutic aspects of these different types. Low grade B cell lymphoma is the most common pulmonary lymphoma. Their development depends on mucosa associated lymphoid tissue. They are most often indolent and present as a chronic alveolar opacity. Their prognosis is excellent and the modalities of treatment are discussed (no therapy, surgery or monochemotherapy). High grade B cell pulmonary lymphomas are much rarer and may result from the transformation of a low grade lymphoma or arise in a particular situation such as imunodepression. Their prognosis is poor and the therapeutic possibilities depend most often on the underlying disease. The presence of lymphomatoid granulomatosis in this group of pulmonary lymphomas is debatable. The demonstration of a clonal character of this proliferation is practically never obtained and there is often extra pulmonary disease. The prognosis of this type of illness is extremely variable because certain studies have shown a cure using corticosteroids and cyclophosphamide whilst others have found that the disease is always fatal in spite of using strong polychemotherapy.
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PMID:[Primary pulmonary lymphoma]. 919 44

Sixty malignant non-Hodgkin's lymphomas originating in the upper aerodigestive tract have been analyzed for their cytologic type, immunophenotype and association with the Epstein-Barr virus (EBV). The majority of these tumors were B-cell lymphomas of blastic cytology (78%) with the exception of lymphomas in the parotid gland. Large B-cell lymphomas were the most frequent encountered in the sinonasal region and Waldeyer's ring. Twelve lymphomas were of T- or T/NK (natural killer)-cell lineage. They were in the nasal cavity and the paranasal sinuses (4), the tonsil (5), and the oral cavity (3). Epstein-Barr sequences were detected in five angiocentric T/NK-lymphomas, one peripheral T-cell lymphoma, one lymphoma of lymphomatoid granulomatosis type, one large B-cell lymphoma, and in a lymphoroliferative disorder in an HIV-positive patient. These results suggest that EBV is not involved in lymphomagenesis of B-cell tumors, but is associated with angiocentric T/NK-cell lymphoma in the upper aerodigestive tract.
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PMID:The Epstein-Barr virus in malignant non-Hodgkin's lymphoma of the upper aerodigestive tract. 927 84

Lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.
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PMID:Lymphomatoid granulomatosis following autologous stem cell transplantation. 1003 54

Few large series compare lymphomas of the nasal cavity with those of the paranasal sinuses. We studied the cases of 58 patients, 34 males and 24 females, aged 7 to 92 years (mean, 57 years), who had lymphoma involving the nasal cavity or paranasal sinuses. Thirty-three patients had diffuse large B-cell lymphoma (DLBCL). Twenty-three were male and 10 were female, with an age range of 7 to 91 years (mean, 63 years); two were HIV-positive. Only 2 of 11 cases tested (one in an HIV-positive patient and one of lymphomatoid granulomatosis type) were Epstein-Barr virus (EBV)-positive. Thirty (91%) involved paranasal sinuses, 10 with nasal involvement, whereas three cases had nasal, but not sinus, involvement. At last follow-up, 16 (67%) were free of disease 7 to 169 months later (mean, 65 months), and 8 (33%) had died of disease 2 to 166 months later (mean, 45 months). Seventeen patients had nasal-type natural killer (NK)/T-cell lymphoma. There were 10 women and 7 men, aged 27 to 78 years (mean, 48 years). Thirteen of 14 were EBV-positive. Sixteen patients had nasal involvement, eight with sinus involvement. Eleven (73%) of 15 were alive and well 6 to 321 months later (mean, 139 months), three (20%) died of lymphoma 1, 11, and 12 months later, and one (7%) is alive with disease. There was one case each of marginal zone B-cell lymphoma, Burkitt's lymphoma, Burkitt-like lymphoma, peripheral T-cell lymphoma of unspecified type, and adult T-cell lymphoma/leukemia. In an additional three cases, the lymphomas were composed predominantly of large cells, but no immunophenotyping could be performed for subclassification. In 19 cases (17 DLBCLs, 1 Burkitt-like lymphoma, and 1 lymphoma of uncertain lineage), presenting symptoms included complaints related to the eyes. In 16 cases (13 DLBCLs, 1 Burkitt-like lymphoma, 1 nasal NK/T-cell lymphoma, and 1 lymphoma of uncertain lineage), the orbit was invaded by lymphoma. In our series, the most common lymphoma to arise in the sinonasal area is DLBCL, followed by nasal NK/T-cell lymphoma. Comparison of these two types of lymphoma showed that lymphomas involving sinuses without nasal involvement were predominantly DLBCLs (20 of 21), whereas nasal cavity lymphomas without sinus involvement were usually NK/T-cell type (8 of 11) (p = 0.000125). Compared with patients with DLBCL, patients with nasal NK/T-cell lymphoma were overall younger, with a lower male-to-female ratio. Lymphomas of B-cell lineage were more likely to be associated with symptoms related to the eyes (p < 0.0005) and to have extension to the orbit (p < 0.01) than were lymphomas of T- or NK-cell lineage. In contrast to results of Asian studies in which nasal NK/T-cell lymphoma has a very poor prognosis, our nasal NK/T-cell lymphomas had an outcome similar to that of DLBCL.
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PMID:Sinonasal lymphoma: a clinicopathologic analysis of 58 cases from the Massachusetts General Hospital. 1055 4

A case of 51 years old woman with 12-years history of rheumatoid arthritis and secondary Sjoegren's syndrome, who developed EBV-associated diffuse large B-cell lymphoma (Lymphomatoid granulomatosis type) involving lungs, lymph nodes and bone marrow was described.
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PMID:[Lung changes during the course of non-Hodgkin's lymphoma in a patient with rhematoid arthritis with secondary Sjogren's syndrome]. 1100 66

Three distinct entities are now covered by the definition of primary pulmonary clonal lymphoid proliferation. The aim of this review is to describe the pathophysiological, diagnostic, prognostic and therapeutic aspects of these three entities. Low-grade pulmonary B-cell lymphoma is the most frequent form of primary pulmonary clonal lymphoid proliferation. It arises from mucosa-associated lymphoid tissue. It is usually indolent and appears in the form of a chronic alveolar opacity. The prognosis is excellent, but treatment is controversial (simple monitoring, surgery or single-agent chemotherapy). High-grade pulmonary B-cell lymphoma is far rarer and usually occurs in individuals with an underlying disorder (e.g. immunodeficiency). The prognosis is poor and therapeutic options depend on the underlying disorder. The inclusion of lymphomatoid granulomatosis in the definition of primary pulmonary lymphomas is controversial. The clonal nature of the proliferation is very rarely demonstrated and extrapulmonary involvement is frequent (upper airways, skin, kidneys, central nervous system, etc.). The prognosis is extremely variable, with some authors reporting complete remission with steroids and cyclophosphamide and others reporting failure of combination chemotherapy.
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PMID:Primary pulmonary lymphoma. 1235 56


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