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)

An anergic patient with arrested Hodgkin's disease developed a cutaneous granulomatous panniculitis, an unusual dermatologic manifestation of this tumor. These granulomatous cutaneous lesions were composed primarily of epithelial cells and were free of necrosis. In contrast, focal necrotic areas were present in the granulomas detected two years previously in the original lymph node biopsy examination.
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PMID:Granulomatous panniculitis and Hodgkin's disease. 672 72

Several subtypes of human malignant lymphomas are known to be highly associated with the Epstein-Barr virus. These include the Burkitt's lymphoma, opportunistic (immune deficiency-associated) lymphoma, nasal T/NK lymphoma, Hodgkin's disease pyothorax-associated lymphoma, cutaneous panniculitis-type lymphoma, and mediastinal large B-cell lymphoma. Improvement of histopathological technology, the demonstration of EBV-encoded small RNAs(EBERs), as well as the molecular virological methods, contributed much in the progression of such EBV-associated lymphomas.
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PMID:[EBV-associated lymphoma]. 904 29

A female patient presenting with B-CLL and coincident eosinophilia-myalgia syndrome (EMS) after ingestion of L-tryptophan is described. The manifestations of EMS disappeared completely during treatment with cyclophosphamide/prednisone. and there was an intermittent clinical remission of CLL with absence of the monoclonal B-cell population. A few years later, the B-CLL relapsed, but without sign and symptoms of EMS. Whereas other eosinophilic syndromes such as eosinophilic fasciitis, panniculitis, or cellulitis Wells have been found to occur in relation to malignant underlying diseases, only a single patient with malignant fibrous histiocytoma following EMS has been described. There are no reports about an increased occurrence of B-CLL or other non-Hodgkin's lymphomas combined with or following EMS or related to L-tryptophan itself. The variant types of eosinophilic syndromes occurring due to malignant disorders, the differentiation from EMS, and the possible association between B-CLL and L-tryptophan-related EMS are discussed.
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PMID:L-tryptophan-related eosinophilia-myalgia syndrome possibly associated with a chronic B-lymphocytic leukemia. 985 50

Mature or peripheral T-cell lymphomas are uncommon, accounting for only 10% to 15% of all non-Hodgkin lymphomas, and their classification has been controversial. In contrast to B-cell lymphomas, cytologic features have not been useful in defining disease entities, and cytologic grade has not been useful in predicting the clinical course. Similarly, many entities of T-cell or NK-cell derivation do not have a specific immunophenotype. Clinical features are of major importance, sometimes more important than the precise cell of orgin, in defining T-cell and NK-cell neoplasms. Most extranodal T-cell/NK-cell lymphomas have a cytotoxic phenotpye. The expression of cytotoxic molecules may predispose to apoptosis by tumor cells and normal bystander cells. Three major categories of extranodal T/NK cell tumors are nasal, intestinal, and subcutaneous panniculitis-like. Hepatosplenic gamma delta T-cell lymphoma is a more systemic disease derived from functionally immature cytotoxic cells. Many extranodal T-cell and NK-cell neoplasms are associated with Epstein-Barr virus (EBV); the association seems site dependent and shows some geographic variation. Tumors resembling any of the 3 prototypes may occur in a variety of extranodal sites. Extranodal T/NK cell lymphomas occur with increased frequency in the setting of immune suppression, especially after organ transplantation.
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PMID:Extranodal peripheral T-cell and NK-cell neoplasms. 989 69

This review covers the diagnosis and management of natural killer and peripheral T-cell lymphomas (PTCL). Problems with PTCL include their rarity, representing usually 10-15% of non-Hodgkin's lymphomas in the Western Hemisphere, morphologic heterogeneity, and lack of immunophenotypic markers for clonality. Additionally, their clinical behavior is variable and may not correlate with morphology. Dr. Kinney gives a general overview of the diagnosis of PTCL and NK cell neoplasms. Emphasis will be placed on extranodal T cell and natural killer (NK) cell lymphomas such as hepatosplenic lymphoma, subcutaneous panniculitis-like lymphoma and nasal/nasal type T/NK-cell lymphoma. The use of ALK gene regulation in the classification of anaplastic large cell lymphoma is also reviewed. Dr. Loughran describes current understanding of the pathogenesis of large granular lymphocyte (LGL) leukemia. The discussion focuses on LGL leukemia as an instructive model of dysregulated apoptosis causing both malignant and autoimmune disease. Current management options and mechanisms of therapeutic response are also described. Dr. Greer addresses whether PTCL should be treated differently from the more common diffuse large B cell lymphomas. He discusses the therapeutic options for anaplastic large cell lymphoma (ALCL), from a conservative approach for primary cutaneous ALCL to combination chemotherapy for the highly chemosensitive ALCL expressing anaplastic lymphoma kinase. He reviews therapy options for the extranodal subtypes of PTCL by drawing from series in adults, pediatrics, dermatology, and the Far East.
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PMID:T cell and NK cell lymphoproliferative disorders. 1172 88

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

Subcutaneous panniculitis-like T-cell lymphoma is a rare tumor of primary cutaneous origin representing far < 1% of all non-Hodgkin's lymphomas. The disease typically follows a distinctive, indolent course of recurrent, self-healing subcutaneous nodules. These nodules mimic lipomas clinically, while histologically resembling a panniculitis. Alternatively, a rapidly progressive course might be seen with subcutaneous nodules accompanied by constitutional symptoms and, in some cases, the development of a potentially fatal hemophagocytic syndrome with significant cytopenia. This tumor is widely regarded as a tumor of CD8+ cytotoxic T cells with the presence of cytotoxic proteins, T-cell-restricted intracellular antigen, and granzyme B commonly demonstrated. A number of modalities have been reported in the treatment of this tumor, with varying degrees of success. In this report, we present 2 cases of subcutaneous panniculitis-like T-cell lymphoma with variable clinical courses. We also review the literature of this unusual lymphoma.
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PMID:Subcutaneous panniculitis-like T-cell lymphoma: presentation of 2 cases and observations. 1252 97

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


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