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

The case report is presented of a patient with gluten sensitive enteropathy who subsequently developed an intestinal non-Hodgkin lymphoma. When steatorrhoea or diarrhoea develops in a patient with abdominal lymphoma, these symptoms are often attributed to progression of the lymphoma or to chemotherapy of the lymphoma. Since there is an established relationship between gluten-sensitive enteropathy and intestinal lymphoma, the differential diagnosis of steatorrhoea or diarrhoea developing in the course of malignant intestinal lymphoma must include gluten-sensitive enteropathy as well. In the investigation for gluten-sensitive enteropathy HLA typing can be used as a screening test in addition to routine malabsorption tests and small bowel biopsy
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PMID:[Gluten-sensitive enteropathy and intestinal non-Hodgkin lymphoma (author's transl)]. 708 Apr 98

From the large amount of data pertaining to a possible relationship between MHC and susceptibility to malignancies in humans, some significant findings emerge. 1. In population studies, HLA-A2 in ALL and A1 in Hodgkin's disease are observed significantly increased in frequency in comparison to the normal controls. Some significant associations with an HLA phenotype are also observed in several cancers. 2. HLA genotyping of familial cases of Hodgkin's disease, with multiplex affected sibs, lead to the conclusions that an excess of HLA identical pairs are observed among the patients. This could indicate a linkage between the susceptibility gene and the HLA region. Another linkage might exist with familial malignant melanoma fitting with a dominant mode of transmission of the trait. These facts strongly support the role of the MHC among the polyfactorial and polygenic determinism of some malignancies. The mechanisms are discussed.
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PMID:[HLA and susceptibility to malignancies]. 712 93

Genetic factors suspected in the etiology of human hemopoietic neoplasia, such as leukemia, lymphoma and multiple myeloma, are reviewed. High incidence of consanguineous marriage was found in parents of familial leukemia in siblings. It was also noted that the age of patients with familial leukemia in children of consanguineous parents was younger than that of cases whose parents were not related. These findings suggest that genetic factors may play an important role in the etiology of familial leukemia in siblings. According to the frequencies of familial aggregations in close relatives, the genetic relationships were supposed to be important in chronic lymphocytic leukemia and acute leukemia, but not in chronic granulocytic leukemia. Increased prevalence of autoimmune diseases in relatives of leukemic patients suggests a possibility of genetic immunodeficiency as a common etiologic factor in both diseases. Immunodeficiency was found in unaffected relatives of patients with familial leukemia and lymphoma. Genetic factors were also suggested by the familial occurrences of multiple myeloma and primary macroglobulinemia, and the incidence of benign monoclonal gammopathy in relatives of patients with these diseases. HLA studies revealed the increased frequencies of A2 in acute lymphocytic leukemia, of B5 and B18 in Hodgkin's disease, and of A5 and B18 in multiple myeloma. From such relationships existing between familial immunodeficiencies and hemopoietic neoplasia, genes regulating the immune responsiveness might be involved in susceptibility to these diseases.
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PMID:[Genetics and hemopoietic neoplasia]. 718 26

Fatal graft-versus-host disease (GVHD) developed in a patient with Hodgkin's disease treated with combined chemotherapy and radiotherapy following the transfusion of 2 U of packed red blood cells. Clinical features of the GVHD included the development of exfoliative dermatitis, progressive hepatic dysfunction, aplastic anemia, and finally progressive fatal pneumonia. GVHD was documented by skin biopsy and chimerism by HLA typing. The HLA phenotype of the patient's skin fibroblasts [A3, Bw44 (w4)/A2, B15 (w4)] was appropriate for parental haplotypes and probably represented her true HLA phenotype. Lymphocytes from the patient (peripheral blood and lymph node biopsy) were of a different HLA phenotype (A3; Bw35, w38, w4, w6; Cw4), which was inappropriate for parental HLA haplotypes but identical to the HLA phenotype of one of the blood donors. The HLA-DR typing of the patient's family and of the blood donor demonstrated that the patient and the donor probably were HLA-DR identical (DRw5/DRw6), although no B lymphocytes could be obtained from the patient for direct DR typing. We are currently irradiating all blood products administered to patients with Hodgkin's disease receiving intensive treatment. Further observations will be necessary to determine whether transfusions to other cancer patients with immunodeficiency states should be restricted to irradiated blood products.
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PMID:Fatal graft-versus-host disease following blood transfusion in Hodgkin's disease documented by HLA typing. 736 71

We report 3 cases of Hodgkin's disease (HD) within a family of seven children. All three children had mixed cellularity type in different stages of the disease (IV-B, III-A, and II-A). The diagnosis was made within a one-month interval. HLA typing shows an identical genotype in the 3 HD patients, which was different from the rest of the family. One of the patients developed an idiopathic thrombocytopenic purpura (ITP) with temporary response to prednisone and MOPP-C chemotherapy that subsided after splenectomy. The HLA identity, in addition to the simultaneous onset of the HD, suggests a combination of both genetic and environmental factors in the pathogenesis of this disease.
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PMID:Simultaneous Hodgkin's disease in three siblings with identical HLA-genotype. 739 49

Analysis of a group of 79 patients with previously untreated Hodgkin's disease, whose HLA phenotypes were determined in 1972-73, shows that patients in a sub-group with the specificities collectively known as Aw19 have significantly poorer survival than patients without these specificities. The degree to which the serologically-detectable HLA antigens assist in determining the prognosis for survival has been analyzed using multiple regression analysis and retrospective stratification. Both approaches indicate that the association of HLA phenotype with survival is not accounted for by correlations between HLA antigens and other know prognostic factors, such as stage, histology, age, and sex. The greatest prognostic value of Aw19 appears to be for patients with relatively unfavorable age (greater than 40 years), stage (III and IV), or histology (lymphocyte depletion, mixed cellularity).
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PMID:The prognostic value of HLA phenotypes in Hodgkin's disease. 742 85

Two sisters had breast cancer at four years and 11 years after diagnosis of Hodgkin's disease. The affected breast tissues had received several hundred rads of scatter radiation during treatment of the lymphoma. Family history revealed breast cancer in a third sister and five other women in the paternal line. Cytogenetic and HLA studies showed no markers of susceptibility to neoplasia. Development of second primary neoplasms of the breast in the two sisters may have resulted from interactions between genetic factors and carcinogenic effects of radiation exposure.
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PMID:Breast cancer after Hodgkin's disease in two sisters. 745 9

The reactive cell population in Hodgkin's disease consists of predominantly CD4+ helper T cells and lacks CD8+ cytotoxic T cells and natural killer cells. This lack of a CD8+ response is surprising in view of the expression of the latent Epstein-Barr viral protein LMP by Reed-Sternberg cells in many cases of Hodgkin's disease, Deficient HLA class I expression would be one possible mechanism to avoid a CD8+ cytotoxic immune response. To test this possibility we studied the expression of HLA class I and II determinants on Reed-Sternberg cells in tissue sections and cell suspensions of Hodgkin's disease. Frozen tissue sections of 40 cases and cytocentrifuge preparations from cell suspensions of 10 lymph nodes involved by Hodgkin's disease were studied with monoclonal antibodies reactive with HLA determinants. As a control frozen tissue sections of two cases of infectious mononucleosis were studied. Careful examination of the tissue sections and subsequently of cytospins of cell suspensions showed that the Reed-Sternberg cells frequently lacked HLA class I but showed strong staining for HLA class II. Absence of HLA class I expression on Reed-Sternberg cells and their variants provides an explanation for the lack of a CD8+ cytotoxic immune response against antigens expressed on Reed-Sternberg cells.
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PMID:Absence of HLA class I expression by Reed-Sternberg cells. 751 95

Several lines of evidence indicate that an impairment of EBV-specific immune responses may contribute to the pathogenesis of Hodgkin's disease (HD). At present, however, it is not clear whether a defective immunity to EBV is a characteristic restricted to EBV-associated HD cases or a more generalized phenomenon, part of the inherent immune deficiency of HD patients. In this study, we have addressed this issue by analyzing EBV-specific responses in infiltrating T lymphocytes (TILs) from one HD biopsy, where the virus was confined to a small proportion of apparently normal lymphocytes. TIL cultures were established using low amounts of recombinant interleukin 2 and in the absence of specific stimulation, conditions that preferentially induce the proliferation of in vivo activated T cells. An EBV-specific cytotoxic component was revealed by the capacity of these TILs to lyse autologous EBV-positive lymphoblastoid cell lines (LCLs) obtained by spontaneous transformation from the lesion but not HLA-mismatched LCLs and autologous phytohemagglutinin blasts. This cytotoxic activity closely resembled that of EBV-specific memory T cells, which may be reactivated from the blood lymphocytes of healthy donors by in vitro stimulation with autologous LCLs. The use of a panel of appropriately HLA-matched B95.8-transformed LCLs as targets in standard 51Cr release assays revealed EBV-specific cytotoxic responses to be restricted mainly through the A11 and B44 HLA alleles with a minor HLA-A26-restricted component. Using autologous fibroblasts infected with recombinant vaccinia viruses expressing the EBV latent antigens, the TIL culture was shown to recognize latent membrane protein 2 and, to a lesser extent, EBV-encoded nuclear antigen 6. In addition, a strong proliferative response was induced by coculture of TILs with autologous but not with allogeneic LCLs or autologous phytohemagglutinin blasts. Six CD4-positive, EBV-specific T-cell clones were isolated by limiting dilution. The study of cytokine mRNA expression, carried out by reverse transcriptase-assisted PCR, revealed that three of these T-cell clones expressed a Th0 phenotype, whereas 1 had a Th2 phenotype. These findings are consistent with the presence in this HD lesion of an ongoing immune response against EBV-carrying cells and suggest that the complex immune deficiency that characterizes HD patients probably does not include a generalized, constitutional defect of EBV-specific T-cell responses.
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PMID:Identification and characterization of an Epstein-Barr virus-specific T-cell response in the pathologic tissue of a patient with Hodgkin's disease. 762 78

Cytotoxic T-lymphocyte (CTL) responses induced by persistent Epstein-Barr virus (EBV) infection in normal B-lymphoid tissues could potentially be directed against EBV-positive malignancies if expression of the relevant viral target proteins is maintained in tumor cells. For malignancies such as nasopharyngeal carcinoma and Hodgkin's disease, this will require CTL targeting against the nuclear antigen EBNA1 or the latent membrane proteins LMP1 and LMP2. Here we analyze in detail a B95.8 EBV-reactivated CTL response which is specific for LMP2 and restricted through a common HLA allele, A2.1. We found that in vitro-reactivated CTL preparations from several A2.1-positive virus-immune donors contained detectable reactivity against A2.1-bearing target cells expressing either LMP2A or the smaller LMP2B protein from recombinant vaccinia virus vectors. Peptide sensitization experiments then mapped the A2.1-restricted response to a single epitope, the nonamer CLGGLLTMV (LMP2A residues 426 to 434), whose sequence accords well with the proposed peptide binding motif for A2.1. Most Caucasian and African virus isolates (whether of type 1 or type 2) were identical in sequence to B95.8 across this LMP2 epitope region, although 2 of 12 such isolates encoded a Leu-->Ile change at epitope position 6. In contrast, most Southeast Asian and New Guinean isolates (whether of type 1 or type 2) constituted a different virus group with a Cys-->Ser mutation at epitope position 1. CTLs raised against the B95.8-encoded epitope were nevertheless able to recognize these variant epitope sequences in the context of A2.1 whether they were provided exogenously as synthetic peptides or generated endogenously in B cells transformed with the variant viruses. A CTL response of this kind could have therapeutic potential in that it is directed against a protein expressed in many EBV-positive malignancies, is reactive across a range of virus isolates, and is restricted through a relatively common HLA allele.
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PMID:HLA A2.1-restricted cytotoxic T cells recognizing a range of Epstein-Barr virus isolates through a defined epitope in latent membrane protein LMP2. 769 72


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