Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It has been recently demonstrated that the Epstein-Barr virus (EBV) can infect human thymocytes and may be involved in the T cell neoplasms, in addition to African Burkitt's lymphoma, nasopharyngeal carcinoma and Hodgkin's disease. Four distinct clinicopathologic categories of EBV-associated T cell malignancies have been recognized. The angiocentric T cell lymphoma or lymphomatoid granulomatosis involving the nose (or midline lethal granuloma) and skin is frequently EBV-associated. The other 3 groups include angioimmunoblastic lymphadenopathy-like lymphoma, node-based T immunoblastic lymphoma which may contain Reed-Sternberg-like giant cells (Hodgkin's-like lymphoma), and T cell lymphoma resembling malignant histiocytosis. Both the CD4 and CD8 T cell subsets, and a hitherto undefined T lineage lacking CD4/CD8 expression have been involved. The common clinical features are prolonged fever, skin lesions, lymphadenopathy, hepatosplenomegaly, and pancytopenia. Serologic assays suggest that a chronic active EBV infection may exist in most of these patients. The EBV genomes appear to proliferate in clonal and episomal form in the neoplastic cells which show expression of latent membrane proteins. Although an indolent local phase may exist, the clinical course is aggressive for most patients with frequent development of drug resistance to conventional chemotherapy. EBV-associated T cell lymphoma constitutes a separate entity of virus-associated human diseases and opens a potential field to investigate the pathogenesis of EBV-associated human malignancies.
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PMID:Clinicopathological spectrum of Epstein-Barr virus-associated T cell malignancies. 133 23

The diagnosis of cutaneous lymphoproliferative diseases is an area of bewildering complexity to many histopathologists. This article concentrates on 'non-mycotic' cutaneous diseases. The 'current state of the diagnostic art' is critically assessed. Cutaneous 'pseudolymphoma' is relegated to the position of an aid-memoire and is not a diagnosis. Inadequacies in the classification of cutaneous lymphoma are discussed and the non-specificity of many histopathological features is highlighted. The status of specific entities is analysed and the contribution of modern investigative techniques in diagnosis is evaluated. This includes cutaneous T-cell lymphomas with a detailed consideration of large cell lymphoma heterogeneity. Cutaneous B-cell diseases are shown to be an unresolved diagnostic maze and the necessity for new clearly defined diagnostic criteria is emphasized. Evidence is presented to show that many cutaneous lymphoproliferative diseases lie on continuous spectra that, initially, are probably antigenically driven, and that a diagnosis is best achieved by a multifaceted approach. This is exemplified by cutaneous diseases that have origins from both B- (cutaneous lymphoid hyperplasia/lymphoma) and T-cells (lymphomatoid papulosis, lymphomatoid granulomatosis and mycosis fungoides). The future diagnostic role of the polymerase chain reaction and cytogenetic analysis is discussed. Intriguingly, recent molecular evidence has shown that lymphomatoid papulosis, cutaneous T-cell lymphoma, CD30 positive large cell lymphoma and Hodgkin's disease can originate from a single T-cell clone and display an identical chromosomal translocation and T-cell receptor rearrangement. Careful clinico-pathological correlation combined with prolonged patient follow-up remains the gold standard for diagnosis.
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PMID:Diagnostic difficulties in 'non-mycotic' cutaneous lymphoproliferative diseases. 139 19

The Epstein-Barr virus (EBV) has been shown to be associated with posttransplant lymphoma, Hodgkin's disease, and T-cell lymphoma, in addition to African Burkitt's lymphoma. In a retrospective study of 56 consecutive cases of T-cell lymphoma, EBV DNA was found by Southern blot and in situ DNA hybridization in 10 (20%) of 50 peripheral T-cell lymphomas, but in none of six cases of T-lymphoblastic lymphoma. Peripheral T-cell lymphomas containing EBV DNA could be subclassified into three categories according to histology and immunophenotypic studies: (1) T-cell lymphoma of the helper phenotype, five cases. Two cases had histologic features resembling angioimmunoblastic lymphadenopathy (AILD). (2) T-cell lymphoma of the cytotoxic/suppressor phenotype, four cases. AILD-like features could also be recognized in two cases. Reed-Sternberg-like giant cells were identified in three cases designated Hodgkin-like T-cell lymphoma. (3) Angiocentric T-cell lymphoma or lymphomatoid granulomatosis in one case, initially affecting the skin and nose; no T-cell subset could be defined. Six of the eight EBV DNA-positive patients tested for serum EBV antibodies had elevated titers of IgG antiviral capsid antigen (greater than 640) and/or early antigen (greater than 10). From combined studies of Southern blot hybridization by using EBV termini fragment probe and in situ DNA hybridization, the EBV genomes appeared to be clonotypically proliferated in the neoplastic T cells. The patients in all three groups usually had prolonged fever preceding the diagnosis, hepatosplenomegaly, an aggressive clinical course, and poor response to chemotherapy; nine died with a median survival of only 8 months. We propose that these EBV-associated aggressive T-cell lymphomas, like human T-cell leukemia/lymphoma virus-positive T-cell lymphoma, have characteristic clinicopathologic features and should be treated as a separate disease entity.
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PMID:Aggressive peripheral T-cell lymphomas containing Epstein-Barr viral DNA: a clinicopathologic and molecular analysis. 184 84

In a prospective study of 42 high-grade lymphomas which were categorized according to the Kiel classification, the clinical significance of immune genotyping was studied. Immunoglobulin (Ig) and T-cell receptor (TCR) gene rearrangements were investigated. In 33 cases the immune genotype confirmed the phenotype. In one case with equivocal phenotype a TCR beta-chain rearrangement proved the T-cell origin of the lymphoma. None of the cases showed a bigenotype. There were eight lymphomas with immunoglobulin and TCR beta-chain and gamma-chain genes in germline configuration, which were divided into a group of immature lymphomas and a group of lymphomas with a more mature phenotype. The immature lymphomas had widespread disease, rapid progression, and favorable prognosis after intensive chemotherapy. The group of T-cell and Ki-1 lymphomas with null-cell genotype was clinically heterogeneous. Three of four cases were secondary lymphomas after lymphomatoid papulosis, lymphomatoid granulomatosis, or Hodgkin's disease. All cases presented with extranodal involvement. Only one of these patients is in continuous remission. In conclusion, the lack of immunoglobulin and TCR beta-chain and gamma-chain gene rearrangements does not exclude the diagnosis of high-grade malignant lymphoma, especially in cases with unusual extranodal involvement. However, the DNA analysis identifies a null-cell genotype subset of high-grade lymphomas which may have clinical significance.
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PMID:Clinical characteristics of high-grade lymphomas with immune genes in germline configuration. 198 55

A review of 40 cases of peripheral T-cell lymphoma identified at our institution between March 1983 and December 1985 revealed a clinically, histologically, and immunologically diverse group of neoplasms that were difficult to classify by conventional histomorphologic criteria for non-Hodgkin's lymphomas. These lymphomas were frequently extranodal at the time of initial manifestation (52%), and their clinical aggressiveness correlated with three major histologic categories--small lymphocytic, diffuse mixed, and large cell. Of the 40 lymphomas, 18 exhibited distinctive histologic features that allowed assignment of these cases into one of four subgroups: (1) angioimmunoblastic lymphadenopathy, (2) lymphomatoid granulomatosis, (3) Hodgkin's-like disease, and (4) Lennert's lymphoma (lymphoepithelioid lymphoma). Study of all our cases that fulfilled the morphologic criteria for lymphomatoid granulomatosis or angioimmunoblastic lymphadenopathy by using immunologic methods for identification of B-cell and T-cell antigens has shown these neoplasms to be peripheral T-cell lymphomas. Therefore, we now consider these earlier proposed entities to be distinct histologic variants of peripheral T-cell lymphoma.
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PMID:Peripheral T-cell lymphomas: histologic, immunohistologic, and clinical characterization. 308 32

Eighty five cases of malignant lymphoma were surveyed for the presence of angioinfiltrative and angiodestructive growth pattern of lymphoid cells with areas of necrosis and granulomatous appearance simulating lymphomatoid granulomatosis (LYG). LYG-like changes were observed in 13 of 85 cases (15%). The most frequent histology was diffuse large cell lymphoma which occurred in 8 cases (4 non-cleaved, 3 cleaved, and one immunoblastic), followed by three diffuse pleomorphic lymphoma, one diffuse medium-sized lymphoma, and one Hodgkin's disease of mixed cellularity. The most frequent involving site was the lung, as well as the soft tissue. Composition of lymphoid infiltrate varied markedly from case to case and from area to area in the same case. One was solely composed of monotonous atypical cells, other pleomorphic cells including benign lymphocytes, plasma cells, leukocytes, and histiocytes with a varied number of atypical reticuloendothelial cells. The latter histologic appearance was just similar to LYG. Veins were more frequently affected than arteries. The present study indicates that, although the angioinfiltrative and angiodestructive growth pattern of atypical lymphoreticular cells with granulomatous appearance is considered necessary for the diagnosis of LYG, it often occurs in malignant lymphoma. Therefore, one should consider the possibility of malignant lymphoma before making the diagnosis of LYG, especially in the specimens taken from the lung and soft tissue.
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PMID:Lymphomatoid granulomatosis-like lesions in malignant lymphoma. 381 7

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

Nineteen cases of possible non-Hodgkin's lymphoma of the lung were studied by conventional morphologic methods and by immunohistochemical methods employing monoclonal antibodies applied to frozen tissue sections. In five of the 19 cases, the original histologic diagnoses were revised after review of the immunologic findings. Problem areas clarified by immunodiagnosis included the differential diagnoses of pseudolymphoma versus small lymphocytic lymphoma (two cases), Hodgkin's disease versus non-Hodgkin's lymphoma (two cases) and non-Hodgkin's lymphoma versus lymphomatoid granulomatosis (one case). Of the seven lymphomas presenting exclusively in the lung without a prior history of lymphoma, three were small lymphocytic, one was diffuse mixed small cleaved and large cell, and three were diffuse large-cell lymphomas. Four of these lymphomas typed as B-cell, two typed as T-cell, and one was of undefined phenotype.
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PMID:Non-Hodgkin's lymphomas of the lung. A study of 19 cases emphasizing the utility of frozen section immunologic studies in differential diagnosis. 387 7

Twenty cases of malignant lymphoma presenting in the lung and 10 cases with secondary pulmonary involvement were studied. All cases shared the feature of prominent vascular infiltration by lymphoid cells, and in the 20 cases presenting with pulmonary involvement, this feature led to confusion with lymphomatoid granulomatosis. Both the primary and secondary lymphomas showed similar histologic features including vascular infiltration, extensive necrosis, and foci of a histologically polymorphous and benign infiltrate. The diagnosis of lymphoma was based on the identification of monomorphous foci of atypical lymphoid cells except in the cases of Hodgkin's disease. The malignant cells were occasionally focal and microscopic and surrounded by an extensive histologically benign infiltrate. Examination of several blocks was often required in such cases before a diagnosis of lymphoma could be made. The clinical and radiologic findings at presentation were nonspecific. Radiologic findings included unilateral or bilateral nodules and infiltrates. The prognosis of the 20 patients who presented with pulmonary lymphoma was poor; half were dead in less than 2 years.
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PMID:Pulmonary lymphomas simulating lymphomatoid granulomatosis. 708 33

Angiocentric lymphoma, which is the malignant counterpart of angiocentric immunoproliferative lesions, comprises a rare group of non-Hodgkin's lymphomas of T-cell origin. It is characterized by marked invasion and destruction of small vessels by lymphomatoid cells, predominantly in the lungs. The prognosis is poor and many patients die within several months. To our knowledge primary involvement of the genitourinary tract has not been previously reported. We report a case of a solitary primary angiocentric lymphoma of the kidney in a patient with the acquired immunodeficiency syndrome. Therapy consisted of nephrectomy without adjuvant treatment. Histological characteristics as well as diagnostic and therapeutic options are discussed.
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PMID:Angiocentric lymphoma of the kidney in the acquired immunodeficiency syndrome. 796 48


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