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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the past decade there have been many advances in our understanding of Hodgkin's disease. Among the most important is the discovery that the Reed-Sternberg cell is a lymphoid cell, in most cases a B cell, and that it is clonal, and thus a true lymphoma, deserving of a name change, to 'Hodgkin's lymphoma' (HL). Based on a combination of immunophenotype and morphology, the R.E.A.L. Classification recognizes two main types of HL: classical types (nodular sclerosis, mixed cellularity, lymphocyte-rich classical HL, and lymphocyte depletion) and nodular lymphocyte predominance type (NLPHL), which probably represent distinct biological entities. 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). Epstein-Barr virus has been detected in approximately 40% of the cases of classical HL, and is clonal, suggesting that this virus may play a role in the pathogenesis of at least some types of HL. The frequency of HL varies in different populations, and the frequency of EBV-positive HL appears to be inversely related to the overall frequency of HL in a given population. Thus, it is possible that its presence may simply reflect the prevalence of EBV-infected B cells in the individual. Despite the advances of the past ten years, many questions remain to be answered, and these will provide the challenges of the next decade.
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PMID:The many faces of Hodgkin's disease around the world: what have we learned from its pathology? 992 37

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

We sought to investigate the clinical characteristics and pathologic features and survival outcome of patients with diffuse large B-cell lymphoma (DLBCL) arising in nodular lymphocyte predominant Hodgkin's disease (NLPHL), since controversy regarding their prognosis exists in the literature. Twenty-one patients with DLBCL arising either concurrently with (n = 7) or subsequent to (n = 14) a diagnosis of NLPHL were identified in the Nebraska Lymphoma Study Group Registry. The clinical and pathologic features of the cases were evaluated, and survival analysis was performed from the time of diagnosis of DLBCL. The median time to the development of DLBCL in those with prior NLPHL was only one year (range, 0.5-24 years). The median age of the patients at the time of diagnosis of DLBCL was 46 years (range, 18-72 years) and the male to female ratio was 17:4. Ten patients presented with nodal DLBCL only, six patients presented with both nodal and extranodal involvement, and five patients presented with only extranodal DLBCL. Eleven patients had limited stage (I/II) disease and 10 had advanced stage (III/IV) disease. The median overall survival (OS) and failure-free survival (FFS) of the entire group was 35 months and 11 months, respectively, and the predicted five-year OS and FFS was 31 and 18%, respectively. There were no significant differences in the survival outcomes between patients with DLBCL arising in NLPHL and age- and sex- matched patients with de novo DLBCL. In conclusion, our findings suggest that patients with DLBCL arising in NLPHL have a prognosis similar to those with de novo DLBCL and should be treated aggressively.
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PMID:Diffuse large B-cell lymphoma arising in nodular lymphocyte predominant hodgkin lymphoma. A report of 21 cases from the Nebraska Lymphoma Study Group. 1473 41

Early acquisition of Epstein-Barr virus (EBV) infection is prevalent in developing countries. We studied infectious mononucleosis (IM) and the subtypes of Hodgkin lymphoma (HL) with the status of EBV infection in Vietnamese children. Among the 46 cases of HL, the male-to-female ratio was 38:8, and the mean age at presentation was 6.6 years. Similar to the subtype distribution in developed countries, cases were classified as nodular sclerosis (NSHL) subtype in 56.5% (n = 26), mixed cellularity (MCHL) in 23.9% (n = 11), lymphocyte-rich classic (LRCHL) in 8.7% (n = 4), lymphocyte depletion (LDHL) subtype in 4.4% (n = 2), and nodular lymphocyte predominance (NLPHL) subtype in 6.5% (n = 3). However, in situ hybridization for EBV-encoded RNA revealed that the tumor cells were positive in 93.2% (41/44) of cases, including all 3 cases of nodular lymphocyte predominance HL. Expression of CD20 on Reed-Sternberg cells could be demonstrated in 17% (7/42) of classic HL. The high incidence of EBV in these cases of HL was correlated with an earlier mean age of presentation of primary EBV infection (ie, IM), at 5.3 years, in this patient population, compared with an average of 15 to 19 years reported in developed countries. This study demonstrates that in an area with an earlier mean age of onset of EBV infection, nearly all cases of pediatric HL, including all histological patterns, may be related to EBV infection. The association of NLPHL with EBV is unusual, and the literature is reviewed and discussed.
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PMID:Epstein-Barr virus is associated with all histological subtypes of Hodgkin lymphoma in Vietnamese children with special emphasis on the entity of lymphocyte predominance subtype. 1608 43

Morphological and immunophenotypical features of Hodgkin's lymphoma with lymphoid predominance are described: a variant with nodular lymphoid predominance (NLPHL) and variants of large-cell lymphoma including mediastinal large B-cell lymphoma, diffuse large B-cell lymphoma rich with T-cells and/or histiocytes. In view of the fact that these lymphomas enter so-called "gray zone" criteria for immunohistochemical differential diagnosis are suggested.
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PMID:[Morphoimmunohistological differential diagnosis between Hodgkin's lymphoma with lymphoid prevalence and large B-cell lymphoma]. 1620 91

Aberrant somatic hypermutation (SHM) has been identified as a mechanism for genomewide instability in diffuse large B-cell lymphoma (DLBCL). To assess whether aberrant SHM plays a role in the molecular pathogenesis of Hodgkin lymphoma (HL), we investigated microdissected neoplastic cells of nodular lymphocyte-predominant HL (NLPHL; n = 10) and classic HL (cHL; n = 9) for the presence of mutations in the 5' sequences of 4 previously identified aberrant SHM targets (PIM1, PAX5, RhoH/TTF, c-MYC). Mutations in one or more genes were detected in 80% of NLPHLs and 55% of cHLs, with 50% and 30% of patients carrying mutations in 2 or more genes, respectively. The most frequently involved protooncogene was PAX5, mutated in 7 of 9 patients with NLPHL and 2 of 9 patients with cHL. In total, 34 mutations were detected in NLPHL (frequency, 1.04/1,000 bp) and 35 were detected in patients with cHL (frequency, 1.92/1,000 bp). Mutations were of somatic origin because they were absent in control T cells and shared most of the features of the immunoglobulin variable (IGV) gene-associated SHM mechanism-ie, single nucleotide substitutions (n = 63) with rare deletions/insertions (n = 6) and a predominance of transitions over transversions with preferential targeting motifs. Our finding that NLPHL and cHL are targeted by aberrant SHM, as is DLBCL, suggests that these lymphomas may share common molecular pathogenetic events.
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PMID:Aberrant somatic hypermutation in tumor cells of nodular-lymphocyte-predominant and classic Hodgkin lymphoma. 1661 47

This study was aimed to investigate the relationship between immunophenotype of the background lymphocytes and histological subtype of Hodgkin's lymphoma (HL), and its significance. The relative protein expressions of background lymphocytes were detected in 37 HL specimens on the basis of instant-rapid MaxVision(TM) immunohistochemical method and assessed quantitatively with image analysis software IPP6.0. The adoptive antibody included anti-CD3/CD45RO, anti-CD20/CD79a, anti-CD4, anti-CD8, anti-GrB, anti-TIA-1. The results indicated that out of 37 cases 4 were NLPHL, 33 were CHL including 6 of MCHL, 14 of NSHL, 13 of LRHL. In addition, 10 cases (1 was NLPHL, 4 were NSHL, 5 were LRHL) were involved in the analysis of T/B ratios. The ratio of T/B in NLPHL was 0.28 +/- 0.07, in CHL 4.34 +/- 2.45 (p = 0.001), in CHL the ratio was LRHL > NSHL > MCHL (p = 0.649); CD4(+)/CD8(+) ratio in NLPHL was 4.55 +/- 1.28, in CHL 4.10 +/- 1.50 (p = 0.574), in CHL it was MCHL > NSHL > LRHL (p = 0.037); GrB(+)/TIA-1(+) ratio in NLPHL was 0.71 +/- 0.57, in CHL 0.74 +/- 0.39, it was MCHL > NSHL > LRHL (p > 0.05). It is conduced that immune cell composition of the diagnostic HL lymph node represents the immune microenvironment. It is different between NLPHL and CHL in terms of the T- and B-lymphocyte distribution. NLPHL is of unique feature. The subtypes of CHL are of peculiar. The T/B ratio is in order as LRHL > NSHL > MCHL, but CD4(+)/CD8(+) and GrB(+)/TIA-1(+) ratios are in the opposite order. Combining with prognosis of subtypes of CHL i.e, LRHL > NSHL > MCHL, these data suggest that low ratio of T/B with high ratios of CD4(+)/CD8(+) and GrB(+)/TIA-1(+) may represent biological markers predicting an unfavorable outcome of CHL subtypes.
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PMID:[Relationship between lymphocyte immunophenotypes and histologic subtypes of Hodgkin's lymphoma and its significance]. 1969 23

A 25-year-old male patient presented to our Ear, Nose and Throat clinic with a history of nausea, vomiting, headache, vertigo and weight loss of 5 kg over the preceding 3 months. An enlarged cervical lymph node was detected at clinical examination. Lymph node biopsy showed nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL, nodular paragranuloma). Because of the neurological symptoms a cerebral MRI scan was performed and revealed an intense perivascular, bilateral, contrast-medium enhancing lesion of the temporal lobes suggestive of cerebral vasculitis. Cerebrospinal fluid analysis showed an increased number of mononuclear cells, but there was no indication for neurotropic viral or bacterial infections. EEG revealed a left temporal epileptic focus, and anti-epileptic therapy was initiated. NLPHL was treated with 2 cycles of ABVD chemotherapy and 20 Gy involved-field radiotherapy. Steroid therapy (prednisone 100 mg q.d.) for the presumed paraneoplastic neurological manifestation was started 1 week before chemotherapy and led to the rapid disappearance of complaints. Because of renewed onset of nausea and vertigo after 3 weeks of treatment with ABVD chemotherapy and 4 weeks of treatment with steroids, a follow-up brain MRI and EEG were performed and demonstrated complete disappearance of the 'vasculitic' changes without additional pathologic findings. Five months after therapy, the patient is without neurological symptoms and a PET-CT showed a complete remission. This case is a unique example of paraneoplastic central nervous system (CNS) involvement in a patient with newly diagnosed NLPHL. We present a review of the literature on paraneoplastic CNS symptoms in Hodgkin's lymphoma.
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PMID:Hodgkin's Lymphoma and Paraneoplastic Phenomena in the Central Nervous System: A Case Report and Review of the Literature. 2147 99

T cell/histiocyte-rich large B-cell lymphoma (THRLBCL), originally considered an uncommon variant of Diffuse Large B-Cell Lymphoma (DLBCL), is recognized by the World Health Organisation as a separate clinicopathological entity since 2008. It predominantly affects middle aged men often presenting with advanced stage disease frequently involving spleen, liver and bone marrow at time of diagnosis. According to the WHO, this lymphoma is morphologically characterized by less than 10% of large neoplastic B cells in a background of abundant T cells and frequently histiocytes. Differentiating THRLBCL from other lymphoproliferative disorders such as Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) and Lymphocyte-Rich classical Hodgkin lymphoma (LRcHL) is important from a clinical point of view and can be achieved in most cases, given adequate biopsy specimens, by careful morphological and immunohistochemical evaluation of both the neoplastic cells as well as the nonneoplastic stromal component. According to this WHO definition, THRLBCL is still considered a clinically heterogeneous entity, though it is noted that especially the cases containing numerous histiocytes behave aggressively and show resistance to current therapies for DLBCL. Gene expression profiling studies of THRLBCL provided evidence for a prominent role for this histiocytic component that is important for a tolerogenic host immune response in which they may assist neoplastic cells in escaping the T cell-mediated immune surveillance. Therefore, reserving the diagnosis of THRLBCL to cases containing a large proportion of histiocytes might be relevant, as modulating their activity could provide new therapeutic options.
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PMID:T cell/histiocyte-rich large B-cell lymphoma: an update on its biology and classification. 2208 Nov 5

A small subset of patients with nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHLs) develop a non-Hodgkin lymphoma either concurrently or subsequently, usually T-cell/histiocyte-rich B-cell lymphomas (T/HRBCL), which are subtypes of diffuse large B-cell lymphomas (DLBCL). The standard treatment of DLBCL patients is rituximab-based chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisolone. However, the administration of this chemotherapy regimen to patients with DLBCL arising in NLPHL brings concern about the cardiac toxicity of anthracycline because the majority of these patients had already received anthracycline-based chemotherapy with doxorubicin, bleomycin, vinblastine and dacarbazine at the time of NLPHL. Herein, we report 2 patients with sequential transformation of NLPHL to T/HRBCL. They initially presented with limited-stage NLPHL and subsequently developed T/HRBCL after 16 and 8 months, respectively. At the time of T/HRBCL, they were treated with rituximab, ifosfamide, carboplatin and etoposide, and complete responses were obtained.
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PMID:Rituximab plus Ifosfamide, Carboplatin and Etoposide for T-Cell/Histiocyte-Rich B-Cell Lymphoma Arising in Nodular Lymphocyte-Predominant Hodgkin's Lymphoma. 2294 3


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