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)

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of non-Hodgkin lymphomas with a wide spectrum of clinicopathologic features, and apoptosis mechanisms may have a role in lymphomagenesis. We assessed apoptotic rate (AR) in 112 PTCLs using a tissue microarray developed in our laboratory and a modified terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling assay. The mean AR was 1.47% +/- 1.38% for the entire group of PTCLs (range, 0.06%-5.15%), and AR varied significantly among different tumor types. In mycosis fungoides, the mean AR was 0.74%; angioimmunoblastic T-cell lymphoma, 1.02%; PTCL, not otherwise specified, 1.38%; cutaneous anaplastic large cell lymphoma (ALCL), 1.41%; anaplastic lymphoma kinase protein (ALK)-negative ALCL, 1.43%; extranodal natural killer/T-cell lymphoma of nasal type, 2.04%; ALK-positive ALCL, 2.95%; and enteropathy-type T-cell lymphoma, 3.06%. Mean AR was higher in PTCL with large cell vs small/medium cell morphologic features (1.66% +/- 1.1% vs 0.99% +/- 1.0%). In a subset of 33 PTCLs, the tissue microarray results comparedfavorably with those obtained in full tissue sections. We conclude that the highest ARs in PTCLs are found in enteropathy-type T-cell lymphoma and ALK-positive ALCL, and that AR can be assessed reliably by using a tissue microarray.
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PMID:Apoptotic rate in peripheral T-cell lymphomas. A study using a tissue microarray with validation on full tissue sections. 1221 74

T-cell non-Hodgkin's lymphomas (NHL) represent approximately 10-15% of all lymphomas diagnosed in Western countries. Significant progress has been made over the last 2 decades in defining non-random chromosomal abnormalities. Cytogenetic and molecular analyses have enhanced diagnostic capabilities as well as improved classification and prognostication for T-cell NHL. Gamma-delta T-cell receptor (TCR) clonality now represents the more common TCR rearrangement in subcutaneous panniculitis-like T-cell lymphoma (SCPTCL), hepatosplenic T-cell lymphoma (HSTCL), extranodal NK/T-cell lymphoma, nasal type and enteropathy-type intestinal T-cell lymphoma (EITCL). Non-random, recurrent chromosome abnormalities such as isochrome 7 with HSTCL, complex karyotypes with peripheral T-cell lymphoma, not otherwise characterized (PTCL-NOC), trisomies 3 and 5 with angioimmunoblastic lymphoma (AIL) and t(2;5) with systemic anaplastic T-cell lymphoma have been recognized. Furthermore, identification of relevant protooncogenes and tumor suppressor genes involved in the pathogenesis of T-cell NHL such as the NPM/ALK fusion protein, p53, cyclin dependent kinase inhibitors (p15, p16, p21) and EBV as well as their interplay with the various regulatory pathways of cell cycle progression and apoptosis represent potential candidates for molecular-based therapy. This review presents a detailed analysis of the molecular and genetic perturbations present in mature T-cell lymphomas including discussion of how tumor-specific alterations impact on clinical outcome. Future studies in T-cell NHL are likely to provide additional disease-specific chromosomal translocations and molecular alterations with important translational implications.
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PMID:Molecular etiology of mature T-cell non-Hodgkin's lymphomas. 1245 15

This study was designed to assess the efficacy and safety of gemcitabine, cisplatin and methylprednisolone (GEM-P) for patients with relapsed or refractory Hodgkin's disease (HD) and non-Hodgkin's lymphoma. Twenty-one patients were treated with gemcitabine (1000 mg/m2 d 1, 8 and 15), cisplatin (100 mg/m2 d 15) and methylprednisolone (1000 mg d 1-5) given every 28 d. Of these, 20 patients were evaluable for response. The median age was 38 years (range 17-64 years). Histological subtypes were: nodular sclerosing HD (n = 10), diffuse large B cell (n = 5), T cell-rich B cell (n = 2), follicular (n = 2), mantle cell (n = 1) and enteropathy-associated T-cell lymphoma (n = 1). The median remission duration prior to receiving GEM-P was only 42 d. The overall objective response rate was 80%[95% confidence interval (CI): 56-94%], including five complete and 11 partial responses. GEM-P induced responses in all histological subtypes, primary progressive disease and patients who had received a previous autograft. The only grade 3-4 toxicity was myelosuppression. However, no cases of febrile neutropenia or haemorrhage with thrombocytopenia were encountered. Median survival has not yet been reached and survival probability at 1 year was 60.8% (95% CI: 31.9-80.5%). In conclusion, GEM-P is a novel combination salvage therapy for poor-prognostic primary progressive or multiply relapsed lymphoma patients. It has clinically significant activity with a favourable toxicity profile.
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PMID:Gemcitabine, cisplatin and methylprednisolone chemotherapy (GEM-P) is an effective regimen in patients with poor prognostic primary progressive or multiply relapsed Hodgkin's and non-Hodgkin's lymphoma. 1264 66

Mature or peripheral T-cell lymphomas are uncommon, accounting for only 10% to 15% of all non-Hodgkin's lymphomas. The classification of these neoplasms has been controversial. In contrast to B-cell lymphomas, cytologic features have not been useful in defining disease entities, and cytologic grade has not helped predict the clinical course. Similarly, many entities of T-cell or natural killer (NK) cell derivation do not have a specific immunophenotype. Clinical features are of major importance in defining T-cell and NK cell neoplasms, and in some cases the clinical syndrome, may be more important than the precise cell of origin. The majority of cytotoxic T-cell and NK cell lymphomas arise in extranodal sites. The expression of cytotoxic molecules in these lymphomas may predispose to apoptosis by tumor cells and normal bystander cells. Three major categories of extranodal T/NK cell tumors are recognized in the World Health Organization (WHO) classification: extranodal NK/T, nasal-type; enteropathy-type; and subcutaneous panniculitis-like. Epstein Barr virus (EBV) is closely linked to nasal NK/T-cell lymphoma, but shows geographic and racial variations in other subtypes. Tumors resembling the prototype of nasal NK/T-cell lymphoma occur in a variety of extranodal sites, and are referred to as nasal-type. Hepatosplenic T-cell lymphoma is a more systemic disease derived from functionally immature cytotoxic cells, usually gammadelta T-cell origin. Cytotoxic T-cell lymphomas of mature gammadelta T-cell origin most often arise in mucocutaneous sites, and may resemble the prototypes of extranodal T/NK cell lymphoma: nasal, enteropathy-associated, and panniculitis-like. Cytotoxic T/NK cell lymphomas occur with increased frequency in the setting of immune suppression, especially following organ transplantation. The nodal T-cell lymphoma most often exhibiting a cytotoxic immunophenotype is anaplastic large cell lymphoma (ALCL). Primary cutaneous ALCL frequently but not invariably expresses cytotoxic molecules. While the majority of extranodal neoplasms are derived from innate immune effector cells of NK cell and T-cell origin (gammadelta greater than alphabeta), most nodal cytotoxic T-cell lymphomas probably belong to the adaptive immune system. Studies of these neoplasms may assist in unraveling the diversity of their normal counterparts.
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PMID:Classification of cytotoxic T-cell and natural killer cell lymphomas. 1287 66

The bcl-3 gene at chromosome 19q13 encodes a member of the IkappaB family involved in regulating the NFkappaB pathway. Originally identified by its involvement in the rare t(14:19)(q32;q13), BCL-3 expression has never been analyzed in a wide variety of lymphomas. We assessed BCL-3 expression in 353 cases of non-Hodgkin lymphoma and Hodgkin lymphoma using formalin-fixed, paraffin-embedded tissue specimens, a monoclonal antibody specific for BCL-3, and immunohistochemical methods. Of 172 B-cell lymphomas, 10 (6%) were positive for BCL-3, including six of 23 (26%) diffuse large B-cell lymphoma, one of 17 (6%) small lymphocytic lymphoma, one of 26 (4%) follicular lymphoma, and two of 49 (4%) mantle cell lymphoma. All other B-cell neoplasms were negative, including marginal zone lymphoma (n=24, 11 extranodal, nine nodal, four splenic), Burkitt lymphoma (n=10), lymphoplasmacytic lymphoma (n=10), lymphoblastic lymphoma (n=8), and plasmacytoma (n=5). Of 111 T/NK-cell lymphomas, 25 (23%) were positive for BCL-3, including 13 of 40 (32%) anaplastic large-cell lymphoma, three of 10 (30%) angioimmunoblastic T-cell lymphoma, two of eight (25%) extranodal NK/T-cell lymphoma of nasal type, three of 12 (25%) mycosis fungoides, one of five (20%) enteropathy-type T-cell lymphoma, and two of 21 (10%) peripheral T-cell lymphoma unspecified. All other T-cell neoplasms were negative, including lymphoblastic lymphoma (n=6), prolymphocytic leukemia (n=6), and subcutaneous panniculitis-like T-cell lymphoma (n=3). Of 70 Hodgkin lymphomas, of all types, 29 (41%) were positive for BCL-3. The relatively high frequency of BCL-3 expression in some non-Hodgkin and Hodgkin lymphoma types raises the possibility that BCL-3 is involved in the pathogenesis of these tumors, and may be a target of new therapies.
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PMID:Immunohistochemical detection of BCL-3 in lymphoid neoplasms: a survey of 353 cases. 1510 10

Using immunohistochemical methods, we evaluated zeta-associated protein (ZAP)-70 expression in 341 cases of non-Hodgkin and Hodgkin lymphoma. In B-cell NHL, ZAP-70 was positive in five of six (83%) precursor B-lymphoblastic lymphoma, 11 of 37 (30%) chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), five of 39 (13%) mantle cell lymphoma, one of 12 (8%) Burkitt lymphoma, and one of 12 (8%) nodal marginal zone B-cell lymphoma. In 22 cases of CLL/SLL, seven of nine (78%) with unmutated IgVH genes expressed ZAP-70, compared with one of 13 (8%) with mutated IgVH genes (P=0.0015 Fisher's exact test). ZAP-70 expression was not detected in diffuse large B-cell lymphoma (n=26), extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (n=24), follicular lymphoma (n=21), plasma cell myeloma/plasmacytoma (n=10), lymphoplasmacytic lymphoma (n=10), or splenic marginal zone lymphoma (n=6). In T/NK-cell NHL, ZAP-70 was positive in all extranodal natural killer (NK) / T-cell lymphoma, nasal-type (n=6) and enteropathy-type T-cell lymphoma (n=4), four of five (80%) subcutaneous panniculitis-like T-cell lymphoma, six of eight (75%) mycosis fungoides, three of five (60%) precursor T-lymphoblastic lymphoma, 10 of 17 (59%) peripheral T-cell lymphoma, two of four (50%) blastic NK-cell lymphoma, one of three (33%) T-cell prolymphocytic leukemia, 13 of 52 (25%) anaplastic large cell lymphoma, and one of six (17%) angioimmunoblastic T-cell lymphoma. Seven of 12 (58%) cutaneous CD30-positive lymphoproliferative disorders were also ZAP-70-positive. In Hodgkin lymphoma, ZAP-70 was negative in neoplastic cells in all cases tested. ZAP-70 staining in B-cell lymphomas and reactive T cells was predominantly nuclear with variable cytoplasmic staining. By contrast, ZAP-70 staining in T/NK-cell lymphomas was heterogeneous, and a shift from predominantly nuclear to predominantly cytoplasmic staining was observed, particularly in those neoplasms with high-grade morphology. In summary, ZAP-70 is expressed by many lymphoma types, correlates with immunoglobulin heavy-chain variable region gene mutational status in CLL/SLL, and can be detected reliably using immunohistochemical methods.
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PMID:Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. 1513 73

The aim of the present review was to summarize the current evidence on the role of ultrasonography (US) and doppler-US in the diagnosis of celiac disease. Several ultrasonographic signs have been reported in the association with celiac disease in studies using real-time US. Firstly, case control studies identified some of these US signs and then in a prospective series some of these parameters, due to their high specificity, have been shown to be of value in confirming CD diagnosis, whereas others, due to their high sensitivity, have been demonstrated to be useful in excluding the presence of the disease. The pattern of splanchnic circulation in CD have extensively been investigated by several studies all of which reported similar results and identified a hyperdynamic mesenteric circulation that reverts to normal values after successful a gluten-free regimen. The last part of this review will deal with the possible role of US in identifying the most severe and common intestinal complication of CD, i.e. the enteropathy-associated T cell non-Hodgkin lymphoma.
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PMID:The role of ultrasonography in patients with celiac disease. 1653 37

The clinicopathological characteristics of malignant lymphomas vary according to geography. The purpose of this study is to determine the distribution and clinicopathological characteristics of malignant lymphomas in Taiwan. Archival tissue from 598 malignant lymphomas during the period of 1995-2002 was retrieved. They were reclassified according to the World Health Organization classification system. Clinical data, including age, gender, clinical staging, and follow-up, were scrutinized. There were 330 males and 268 females. The median age at onset of disease was 56 years for B-cell lymphoma (BCL), 50 years for T/NK-cell lymphoma (TCL), and 26 years for Hodgkin's lymphoma (HL). BCL accounted for 80.6%, TCL for 12.4%, and HL for 7%. The major subtypes of non-HL were diffuse large B-cell lymphoma, follicular lymphoma, plasma cell myeloma, marginal zone lymphoma of mucosa-associated lymphoid tissue type, mantle cell lymphoma, unspecified peripheral TCL, and nasal type T/NK-cell lymphoma. Nodular sclerosing subtype was the most common in HL. The frequencies of TCL and HL were relatively low. For histological subtype, enteropathy-type TCL and primary bone marrow HL had higher frequency and poorer prognosis. The 5-year overall survival of BCL, TCL, and HL was 58.9, 34.7, and 83.5%, respectively. To the best of our knowledge, this is the largest series study of malignant lymphoma in Taiwan. Immunophenotype, histological subtype, and clinical stage play significant roles in prognosis (P < 0.05).
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PMID:Clinicopathological analysis of 598 malignant lymphomas in Taiwan: seven-year experience in a single institution. 1682 25

The pathogenesis of mature T-cell non-Hodgkin lymphomas (T-NHLs) is poorly understood. Analogous to B-cell lymphomas, in which the immunoglobulin (IgH) receptor loci are frequently targeted by chromosomal translocations, the T-cell receptor (TCR) gene loci are affected by translocations in a subset of precursor T-cell malignancies. In a large-scale analysis of 245 paraffin-embedded mature T-NHLs, arranged in a tissue microarray format and using improved FISH assays for the detection of breakpoints in the TCRalpha/delta, TCRbeta, and TCRgamma loci, we provide evidence that mature T-NHLs other than T-cell prolymphocytic leukaemia (T-PLL) also occasionally show a chromosomal rearrangement that involves the TCRalpha/delta locus. In particular, one peripheral T-cell lymphoma (not otherwise specified, NOS) with the morphological variant of Lennert lymphoma displayed a chromosomal translocation t(14;19) involving the TCRalpha/delta and the BCL3 loci. A second case, an angio-immunoblastic T-cell lymphoma (AILT), carried an inv(14)(q11q32) affecting the TCRalpha/delta and IgH loci. FISH signal constellations as well as concomitant comparative genomic hybridization (CGH) data were also suggestive of the occurrence of an isochromosome 7, previously described to be pathognomonic for hepatosplenic T-cell lymphomas, in rare cases of enteropathy-type T-cell lymphoma.
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PMID:Tissue microarray-based screening for chromosomal breakpoints affecting the T-cell receptor gene loci in mature T-cell lymphomas. 1758 37

The gastrointestinal (GI) tract contains the largest collection of lymphocytes anywhere in the body. GI lymphoma may arise at any site in the GI tract but typically involves the stomach and small bowel in cases of systemic disease. Most cases are non-Hodgkin B-cell type. Enteropathy-associated T cell lymphoma can complicate celiac disease. Less commonly, lymphoma may originate in the GI tract without systemic involvement. This sometimes occurs in response to chronic infections. This article discusses the role of imaging in detecting and staging GI tract lymphomas, using fluoroscopy and cross-sectional imaging, primarily CT.
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PMID:Imaging of gastrointestinal lymphoma. 1861 82


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