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)

Several reports of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) and of coexisting or subsequent Hodgkin's disease (HD) have raised the question how these two disorders are related. The authors have identified eight new cases of B-cell low-grade lymphoproliferative disorders (LGLPD) and HD. Six of these cases were similar to those previously reported on by others in that the HD were mixed cellularity, nodular sclerosing, and lymphocyte depleted subtypes. The morphology in these cases was typical of HD, as was the immunohistochemical profile. However, the two remaining cases were notable in that the HD was of the nodular lymphocyte predominant type (NLPHD). To our knowledge, this association has not been well documented previously. In the two cases in this study, CLL and NLPHD were found simultaneously when each patient presented with lymphadenopathy and a lymphocytosis that was comprised of small monoclonal B lymphocytes coexpressing CD5. Lymph node biopsies in each case revealed typical NLPHD, with large, indistinct nodules containing scattered lymphocytic-histiocytic (L&H) cells. Focal, but distinct areas of CLL/SLL were also present. Immunostaining of the lymph node biopsy specimens showed the L&H cells to be CD20- and CD45 positive, and to lack CD15 or evidence of light chain restriction. In one of these patients, a NLPHD-associated large cell lymphoma developed 8 months later. The large cells were CD20- and CD45 positive, with lambda light chain restriction. In contrast, the original CLL cells in this patient expressed kappa light chains. This report indicates that LGLPD can be associated with all subtypes of HD, including the NLP type. The discordant light chain restriction between the CLL and the NLPHD-associated large cell lymphoma in one of these cases indicates that the CLL and HD were probably not derived from the same clone.
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PMID:Hodgkin's disease associated with chronic lymphocytic leukemia. Eight additional cases, including two of the nodular lymphocyte predominant type. 772 47

We evaluated the presenting clinical characteristics and outcome of 68 patients with nodular lymphocyte predominance Hodgkin's disease (NLP-HD), in order to delineate the pattern of evolution of the disease. The male to female ratio was 46/22 and median age 35 yrs (range, 14-86). Eight patients had a history of benign hyperplasia on lymph node biopsies performed 6 to 36 months before the diagnosis of NLP-HD. Early stage disease accounted for 75% of cases. One patient had a coexistent non-Hodgkin's lymphoma (NHL). Treatment was as follows: radiotherapy in 26, chemotherapy in 23, combined modality in 19. CR rate was 93% (63/68). 18 patients relapsed as HD and 5 developed NHL. The cumulative risk of NHL was 9% at 10 yrs. During remission, 4 patients had 5 episodes of follicular hyperplasia histologically documented. Overall survival rate was 71% at 10 yrs and and 63% at 15 yrs. Freedom from progression (FFP) declined from 67% at 5 yrs to 45% at 10 yrs, because of late relapses. Localized disease predicted for a better FFP (p = 0.01), but was not associated with a reduced risk of recurrence over time. NLP-HD is characterized by an indolent course with a constant pattern of relapse over time, also in patients with early stage disease at diagnosis. In addition to relapse as NLP-HD, patients may evolve into a NHL or develop benign lymph nodal hyperplasia. Careful long-term follow up is needed for these patients.
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PMID:Nodular lymphocyte predominance Hodgkin's disease: long-term observation reveals a continuous pattern of recurrence. 932 99

Hodgkin's lymphoma is characterized by the combination of Reed-Sternberg (R-S) cells and a prominent inflammatory cell infiltrate. One of the intriguing questions regarding this disease is what is causing the influx of T lymphocytes into the involved tissues. We applied the serial analysis of gene expression (SAGE) technique on the Hodgkin's lymphoma-derived cell line L428 and on an Epstein-Barr virus (EBV)-transformed lymphoblastoid B-cell line. A frequently expressed tag in L428 corresponded to the T-cell-directed CC chemokine TARC. Reverse transcription polymerase chain reaction analyses demonstrated expression of TARC in nodular sclerosis (NS) and mixed cellularity (MC) classical Hodgkin's lymphomas but not in NLP Hodgkin's lymphoma, anaplastic large-cell lymphomas, and large-B-cell lymphomas with CD30 positivity. Two of five cases of T-cell-rich B-cell lymphoma (TCRBCL) were TARC positive. RNA in situ hybridization (ISH) showed a strong signal for TARC in the cytoplasm of R-S cells, and immunohistochemical staining confirmed the presence of the TARC protein in the R-S cells of NS and MC Hodgkin's lymphomas. The lymphocytic and histiocytic (L&H)-type cells of nodular lymphocyte predominance Hodgkin's lymphoma and the neoplastic cells of non-Hodgkin's lymphomas with the exception of two cases of TCRBCL did not stain for TARC. TARC is known to bind to the CCR4 receptor, which is expressed on activated Th2 lymphocytes. The immunophenotype of lymphocytes surrounding R-S cells is indeed Th2-like, and by RNA ISH these lymphocytes showed a positive signal for the chemokine receptor CCR4. The findings suggest that production of TARC by the R-S cells may explain the characteristic T-cell infiltrate in classical Hodgkin's lymphoma.
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PMID:High expression of the CC chemokine TARC in Reed-Sternberg cells. A possible explanation for the characteristic T-cell infiltratein Hodgkin's lymphoma. 1036 93

Much has been learned in the past decade from the study of tissues involved by the disorder long known as Hodgkin's disease. Two important discoveries have prompted changes in classification and nomenclature: first, the recognition that there are two distinct lymphomas encompassed within this category (classical and nodular lymphocyte-predominant [NLP] types), and second, the discovery that the Reed-Sternberg (RS) cells in most cases are monoclonal B cells. Thus "Hodgkin's disease" comprises two distinct lymphomas, deserving of a name change, to "Hodgkin's lymphomas" (HLs). The immunophenotype and genetic features of both classical HL and NLPHL have been defined. These are useful in the subclassification of HL and in distinguishing HL from two recently described, aggressive lymphomas that were in the past often diagnosed as HL: anaplastic large-cell lymphoma, T-cell type (ALCL), and T-cell/histiocyte-rich large B-cell lymphoma (T/HRBCL). Despite the advances of recent years, many questions remain to be answered, and these will provide the challenges of the next decade.
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PMID:Hodgkin's lymphomas: classification, diagnosis, and grading. 1046 21

Biological and clinical studies have shown that Hodgkin's disease (HD) can be divided into two major categories, termed nodular lymphocyte predominance HD (NLP HD) and classic HD (CHD). Within CHD four subtypes have been distinguished: nodular sclerosis, mixed cellularity, lymphocyte rich and lymphocyte depletion. To refine the histogenesis of the pathological spectrum of HD, 75 CHD and 13 NLP HD were analysed for the expression pattern of MUM1/IRF4 (Multiple Myeloma-1/Interferon Regulatory Factor-4), a lymphocyte-specific member of the IRF family, that is expressed by late centrocytes and post-germinal centre (GC) B cells. MUM1 reacted with Hodgkin's and Reed-Sternberg (HRS) cells of all CHD cases (75/75 cases), with a moderate to strong staining intensity. Conversely, lymphocyte and histiocyte (L & H) cells, the putative tumour cells of NLP HD, were negative for MUM-1 expression (9/13 cases) or displayed a weak reactivity for the antigen in < 10% neoplastic cells (4/13 cases). With respect to HD microenvironment, NLP HD displayed numerous MUM1-positive T lymphocytes located in close proximity to L & H cells whereas, in CHD, MUM1-positive T lymphocytes appeared to be distributed randomly with no specific relationship with HRS cells. Overall, this study shows that MUM1 expression differs in L & H cells versus HRS cells, corroborating the notion that NLP HD and CHD represent different stages of B-cell differentiation. As MUM1-positive T lymphocytes form rosettes around tumour cells of NLP HD, but not of CHD, these data point also to differences in the microenvironment of NLP HD and CHD, and postulate an interactive role of MUM1-positive T lymphocytes with L & H cells.
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PMID:Expression pattern of MUM1/IRF4 in the spectrum of pathology of Hodgkin's disease. 1197 19

Hodgkin lymphoma (HL) is characterized by a minority of neoplastic cells, the so-called Reed-Sternberg (RS) cells and a vast majority of reactive cells. RS cells produce chemokines that can attract subsets of peripheral blood cells into HL tissues. To gain insight in the chemokines involved in HL, 16 chemokines were selected based on their ability to recruit different subsets of cells. Five HL, 5 non-HL-derived cell lines, 22 HL, 5 non-HL and 3 control tissues were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). Products for 13 of these 16 chemokines were detected in 1 or more of the cell lines tested. No or only very faint signals were obtained in HL for CXCL12, CCL7 and CCL8, but CXCL10, CCL5, CCL13, CCL17 and CCL22 were highly or differentially expressed in HL cell lines and tissues. Immunohistochemistry was performed with antibodies reactive with the latter 5 chemokines on paraffin sections of 21 cases of HL. CCL17 and CCL22 had the highest signals in RS cells at gene expression and at protein levels. CCL17 was specific for the classic HL subtypes, whereas CCL22 also had low signals in NLP samples, as well as in some non-HL. CXCL10 was expressed in a large proportion of HL cases with a predominant expression in EBV-positive cases. The results indicate that RS cells produce a complex pattern of chemokines that are involved in the recruitment of reactive cells and contribute to the paradox of an extensive but ineffective host immune response.
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PMID:Common and differential chemokine expression patterns in rs cells of NLP, EBV positive and negative classical Hodgkin lymphomas. 1211 99

Autoimmune Lymphoproliferative Syndrome (ALPS) is generally the result of a mutation in genes associated with apoptosis, like Fas, Fas ligand, Casp 8 and Casp 10. As a result, the normal homeostasis of T- and B-lymphocytes is disturbed and a proliferation of polyclonal T lymphocytes occurs. This leads to hepatosplenomegaly and lymphadenopathy and in most patients also to autoimmune phenomena like anemia and thrombocytopenia. The proliferating T cells are TCRalphabeta and/or TCRgammadelta positive but lack both CD4 and CD8. Hence they are termed double negative (DN) T cells. In addition, there is an increase of CD5 positive B cells. Individuals with germline mutations in the Fas gene have a high risk to develop non Hodgkin lymphomas (x 14) as well as Hodgkin lymphomas (x 51), in particular NLP Hodgkin lymphoma. Somatic mutations of Fas are frequently acquired during the normal germinal center reaction. Non Hodgkin lymphomas carry somatic mutations of the Fas gene in 11% and of the Casp 10 gene in 14.5% of the patients. In Hodgkin lymphomas, Fas mutations can be demonstrated in Reed-Sternberg cells in 10-20% of the patients. These data implicate a role for Fas-mediated apoptosis in preventing lymphomas. Inherited defects in receptor-mediated lymphocyte apoptosis represent a risk factor for lymphomas and somatic mutations of these genes may also play a role in the development and/or progression of lymphomas.
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PMID:Development of lymphoma in Autoimmune Lymphoproliferative Syndrome (ALPS) and its relationship to Fas gene mutations. 1516 Sep 2

Aim: To present the case of a 57-year-old male with progressive bilateral proptosis. Material and method: The patient presented with bilateral proptosis and strength deficiency on the upper limbs. During hospitalization, the progression was unfavorable; proptosis progressed, causing a severe loss of vision in the left eye (from 0.8 Snellen to NLP). Results: The imagistic investigation revealed bilateral infiltration of the orbits, infiltrative lesions to the mediastinum and the abdomen. The patient was referred to neurosurgery for further management. Surgical orbital decompression was performed with biopsy. The histopathological examination revealed non-Hodgkin small cell lymphoma. Conclusions: Assembling the clinical and paraclinical data we have suspected the possible diagnosis of Erdheim-Chester disease, however, positive diagnosis has not been achieved.
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PMID:A possible case of Erdheim-Chester Disease. 3089 29