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)

In order to further define the factors associated with the observed variations in the Epstein-Barr virus-positive rate in childhood Hodgkin's disease, we have studied the effect of material deprivation (measured by the Townsend score) and ethnic origin on the frequency of Epstein-Barr virus-positivity in 55 cases of childhood Hodgkin's disease, diagnosed between 1981 and 1999, from a multi-ethnic region of the United Kingdom. Epstein-Barr virus status was determined by immunohistochemistry for the Epstein-Barr virus-encoded latent membrane protein-1. 62% of cases were Epstein-Barr virus-positive. Ethnic group was the strongest predictor of Epstein-Barr virus-positivity, with South Asians having a more than 20-fold risk of being Epstein-Barr virus-positive compared with non-South Asians. An increased risk was still present after adjusting for deprivation. Townsend scores were significantly higher (indicating more deprivation) in the Epstein-Barr virus-positive group, particularly in males. The relative risk of Epstein-Barr virus-positivity showed a gradient with increasing Townsend score; the risk being 7-times higher in the most deprived quartile compared with the least deprived group. Although the association between Townsend score and Epstein-Barr virus-positivity was reduced after adjusting for ethnic group, the risk of Epstein-Barr virus-positivity was still 3-times higher in the most deprived compared with the least deprived quartile. In addition, cases having 2 or more siblings were 5-times as likely to be Epstein-Barr virus-positive as those from smaller families. These results provide the first evidence of a strong association between Epstein-Barr virus-positive Hodgkin's disease and South Asian children from the United Kingdom. In addition, deprivation may increase the likelihood of Epstein-Barr virus-positive disease independently of ethnicity.
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PMID:South Asian ethnicity and material deprivation increase the risk of Epstein-Barr virus infection in childhood Hodgkin's disease. 1148 64

The purpose of the study was to evaluate the incidence of second malignancies in childhood Hodgkin disease treated with COPP and COPP/ABV (replacing mechlorethamine by cyclophosphamide) chemotherapy. In 212 children with Hodgkin disease who were under 14 years of age and treated at Cancer Institute during the 25-year period of 1970-1994, the occurrence of second malignant neoplasms was analyzed as on 31 December 1999. Eighty-two percent attained complete response. The 5- and 10-year overall survival rate was 91 and 83%, respectively. In this interm report 5 cases of second malignancies were documented. All 5 were solid tumors: one each of soft tissue sarcoma, dermatofibrosarcoma, micropapillary carcinoma of thyroid, malignant phylloides tumor of breast, and chondrosarcoma of ilium. All patients had received combination chemotherapy and radiotherapy. Interestingly, all were splenectomized. All these patients had advanced stage of cancer and were 7-14 years of age at the time of diagnosis of first primary Hodgkin disease. It is significant that there were no secondary hematological malignancies. COPP and COPP/ABV are effective therapeutic regimens. The paucity of secondary hematological malignancies is unique in this series and may be attributed to the substitution of nitrogen mustard with cyclophosphamide in the chemotherapy combination. This is an initial observation, and further follow-up is needed for a firm conclusion.
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PMID:Paucity of hematological neoplasia after treatment of Hodgkin disease: observation after long-term follow-up at Cancer Institute, Chennai, south India. 1193 33

Lymphomas are the third most frequent malignancies in childhood. The Hungarian Pediatric Oncology Group was founded in 1971, and since then the same chemotherapeutic protocols have been used in the whole country. In this study we analyzed the data of childhood Hodgkin's lymphoma in Hungary in the last 11 years (1988-1998). We also compared our results with the international (German) data. The incidence of Hodgkin's lymphoma (0-15 years) was 7.1/1,000,000 child/year (the same for non-Hodgkin's lymphoma was 7.5/1,000,000/year); 5.5% of all pediatric malignancies in Hungary). The patients were treated according to the German DAL-HD-82 and 90 protocols. The therapy consisted of 2-6 cytostatic blocks, depending on the stage, followed by involved field irradiation. The overall survival was 94.7+/-2.0% at 5 years and 91.9+/-2.7% at 10 years. These results are very similar to the German data: 94% at 5 years and 93% at 10 years. The good results are due to the well organised network and the uniformed treatment. The results may be ameliorated by using autologous bone marrow transplantation.
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PMID:[Treatment results of childhood Hodgkin's lymphoma in Hungary] 1205 Jun 86

Juan A. del Regato, 1909-1999, was a superb clinician-educator who recognized the radiocurability of Hodgkin's disease but questioned treatment without late effects, particularly in children. The remarkable progress in pediatric Hodgkin's disease today is a tribute to this influential pioneer, who served as a role model to many. Combined modality therapy using low-dose, involved-field radiation and multiagent chemotherapy today results in a 5-year relative survival rate of 94% among American children with Hodgkin's disease. However, several areas hold promise for future advances, including a new pathology classification and biology studies that distinguish classic Hodgkin's disease from other lymphomas; new noninvasive staging techniques, including 18F-fluorodeoxyglucose-positron emission tomography; the definition of risk groups to segregate low-, intermediate-, and high-risk groups on the basis of a prognostic index, facilitating risk-adapted therapy; and myeloablative therapy followed by hematopoietic stem cell transplantation. Currently used for children with relapse, it is associated with a 5-year survival of 65% and should be considered as the initial therapy for high-risk groups. Idiopathic diffuse pulmonary toxicity after autologous transplantation is high among children with an atopic history; thus, atopy should be considered when selecting children appropriate for transplantation. Finally, novel therapies, such as the anti-CD20 antibody, rituximab, may be useful for children with CD20+, lymphocyte-predominant Hodgkin's disease. The universal goal of cure without late effects is realistic for almost all children with Hodgkin's disease today.
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PMID:Pediatric Hodgkin's disease--up, up, and beyond. 1218 68

Although childhood Hodgkin disease is sensitive to the treatment regimens devised for Hodgkin disease in adults, long-term toxicity is enhanced in the developing individual. As a result, there have been dual goals in the design of clinical trials for pediatric Hodgkin disease: 1) to reduce long-term organ injury; and 2) to increase efficacy. Radiation dose and field has been reduced by enhanced reliance on chemotherapy, thus limiting the risks of hypoplasia, hypothyroidism, secondary cancers, and valvular and atherosclerotic heart disease. Multiagent, chemotherapeutic regimens for children have been developed to avoid the risks of sterility, leukemia, and cardiopulmonary toxicity. Newer approaches advocate for early dose intensity to limit cumulative therapy using response-based paradigms. Targeting molecular mechanisms specific for the Reed-Sternberg cell may allow for less toxic and more efficacious treatments in the future.
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PMID:The management of Hodgkin disease in the young child. 1254 64

There are conflicting reports about the involvement of single nucleotide polymorphisms (SNPs) of the ataxia telangiectasia mutated (ATM) gene with cancer, and the consequences of these SNPs for ATM function remain unclear. We therefore sought to identify SNPs of the ATM gene in pediatric Hodgkin disease (HD) and to analyze ATM function in cells from patients with these SNPs. We have identified SNPs of the ATM gene in 5 of 14 children (S1455R, n = 1; H1380Y, n = 1; N1650S, n = 2; and I709I, n = 1). One patient had nonsense-associated altered splicing of the ATM gene. Lymphoblastoid cell lines expressing the S1455R and N1650S exhibited defective ATM-mediated p53 phosphorylation and Chk2 activation; cells expressing the H1380Y exhibited defective c-Abl activation after X-irradiation. Expression of the N1650S in ATM-null fibroblasts conferred only partial hyperradiosensitivity. Furthermore, the introduction of N1650S ATM into U2OS cells, which express wild-type ATM, showed reduced p53-Ser15 phosphorylation, suggesting a dominant-negative effect of the N1650S over the wild-type ATM protein. We conclude that the rare polymorphic variants of the ATM gene that we identified in children with HD encode functionally abnormal proteins, and we discuss the possible genetic risk factors for childhood HD.
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PMID:Identification and characterization of polymorphic variations of the ataxia telangiectasia mutated (ATM) gene in childhood Hodgkin disease. 1296 74

Ataxia telangiectasia is an autosomal recessive disease with a striking predisposition of lymphoid malignancies. ATM mutations have been reported in adult sporadic lymphoma and leukaemia. The aim of this study was to investigate the possible involvement of the ATM gene in the carcinogenesis of Hodgkin disease in children. Tumours were obtained from 23 patients and were subjected to mutation screening and loss of heterozygosity analysis. Eight base substitutions were identified in seven patients. Of them, Y54Y, a silent change, was observed in two patients and a known polymorphism, D1853N, in three patients. Of the other two patients, one harboured a combined genotype P604S/F1463C, identified previously in two patients with Hodgkin lymphoma, and the other a novel missense mutation, V595A. The alterations were present in the germ line, and both had a more aggressive disease. In all, 100 matched normal ethnic controls were screened for these mutations and P604S/F1463C was identified in one healthy control. Loss of heterozygosity was identified in four patients and in three of them it was located centromeric to the ATM gene, and, in one, it spanned a large region, indicating the involvement of other tumour-suppressor genes in this disease. Missense variants of the ATM gene are a rare event in childhood Hodgkin disease.
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PMID:Molecular variants of the ATM gene in Hodgkin's disease in children. 1473 3

Approximately 15% of all cases of childhood classical Hodgkin's disease (HD) express CD20, a B-cell marker associated with immunoglobulin heavy chain rearrangements. Immunoglobulin heavy chain rearrangements in Reed-Sternberg cells could be used to assess minimal residual disease (MRD), as was shown with immunoglobulin heavy chain patient-specific primers (PSPs) in non-Hodgkin's lymphoma. The aim of this study was to analyze pediatric HD for future design of immunoglobulin heavy chain PSP for MRD detection. DNA was extracted from paraffin-embedded tissue from unstained slides of 8 pediatric CD20+ nodular sclerosis HD cases and 10 CD20-nodular sclerosis HD cases. Immunoglobulin heavy chain polymerase chain reaction and sequencing were performed on 16 of 18 cases, which had adequate DNA for further analysis. Sequence analysis from 3 cases (19% of HD cases) demonstrated unique V(D)J regions, which could potentially be used to design PSP. Unique PSPs could be used to assess MRD in advanced-stage HD specimens. Future studies should focus on improved detection and analysis of more cases to identify appropriate specimens in assessing clinical implications of MRD detection.
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PMID:Assessing immunoglobulin heavy chain rearrangements in pediatric CD20-positive and CD20-negative classic Hodgkin's disease. 1563 94

Although treatment of pediatric Hodgkin disease has become highly effective over the past 40 years, a number of patients have developed concerning late effects, such as secondary malignancies. These cancers may occur years to decades after remission and arise in the breast, thyroid, gastrointestinal tract, lung, skin, urogenital tract, and brain. There is also an increased risk of leukemia and non-Hodgkin lymphoma. Etiology and risk factors for each cancer type vary but often include certain chemotherapy agents and radiation dosages. Survivorship also varies but is often poor. The authors examined retrospective analyses of these secondary malignancies and present a summary of these findings. The information may allow clinicians to better monitor childhood Hodgkin disease survivors and reduce mortality.
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PMID:Second malignancy after treatment of pediatric Hodgkin disease. 1565 75

About 1,700 children in the United States are diagnosed yearly with lymphomas; Hodgkin's disease accounts for approximately half of these cases, or 6% of all childhood cancers. Contemporary therapy allows for the achievement of remission in the majority of cases. The fusion of positron emission tomography (PET) with CT provides the most accurate imaging method for disease characterization and treatment response. However, experience with 18F-FDG PET-CT is limited in pediatric Hodgkin's disease. Numerous non-oncologic processes can mimic recurrent or residual tumor. This pictorial addresses mimickers of disease such as uptake in normal structures, infections, transforming germinal canters and effects of therapy on normal tissues. It is essential for radiologists to be familiar with these findings in order to stage disease activity and therapeutic response accurately.
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PMID:18F-FDG-avid sites mimicking active disease in pediatric Hodgkin's. 1565 5


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