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)

The 10 year results of a trial of bimodal treatment of Hodgkin's disease in children with 6 cycles of MOPP and low-dose extended field irradiation, without staging laparotomy, were for 57 children in all stages as follows: survival 85%, relapse-free survival 80%, and survival-free of second relapse 86%. There were three fatal toxic events, two due to viral infection and one to a second malignant tumor (NHL). Three other patients developed a second malignant tumour, and one developed a thyroid adenoma. No patient developed acute leukemia. These results are compared with the results of treatment of surgically staged children by extended field irradiation alone, with bimodal treatment reserved for relapse or advanced disease at diagnosis. Initial bimodal treatment improved the overall 10 year survival free from a second relapse rate by 20% (86% vs. 66%). No major difference in treatment toxicity between these two groups has emerged during the first 10 years of follow-up. We conclude that, except for favourable CS-1 presentations, children with Hodgkin's disease confined to the lymphatic system should be given bimodal treatment, but that the least morbid effective combination remains to be determined.
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PMID:Hodgkin's disease in children: treatment with MOPP and low-dose, extended field irradiation without laparotomy. Late results and toxicity. 235 85

In addition to conventional morphological, histological and immunological marker studies, cells from 150 children with leukemia or non Hodgkin's lymphoma were analysed using the Southern blot hybridization technique to examine immunoglobulin- (Ig) and T-cell receptor (TCR) gene rearrangements. Patients with B-lineage leukemia or NHL demonstrated in 90% an Ig heavy chain gene rearrangement, 6% with an additional light chain kappa gene rearrangement. Combined Ig- with TCR-beta-gene rearrangements were mainly found in patients with common ALL: 19% at first presentation, and 33% in relapse. Moreover, 6 c-ALL patients showed rearrangements in all 3 gene loci (JH-, Ck- and TCR). Based on the developmental hierarchy of Ig- and TCR gene rearrangements it was possible to further subclassify c-ALL into different stages of B cell development. No correlation could be established between the different constellations of gene rearrangements, the number of rearranged fragments and the course of illness. All patients with T-lineage leukemia or NHL demonstrated TCR rearrangements of the beta-, g- and delta-gene loci, two with an additional Ig gene rearrangement. These data confirm recent reports indicating that immunoglobulin heavy chain gene rearrangements are not restricted to B-lineage neoplasms. Furthermore, non-germline configuration was found in tumor cells of every patient with AUL, O-ALL and AHL, permitting a classification to B- or T-cell lineage. Noteworthy is that every AML patient with Ig- and/or TCR gene rearrangements showed a poor or non-response towards therapy. Specimens of individual patients with differently involved tissues at diagnosis always showed an identical rearrangement. The intensity depended on the number of infiltrating blast cells.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical applications of the study of immunoglobulin and T-cell receptor gene rearrangements in acute leukemia and non-Hodgkin's lymphoma in children]. 239 11

In a multicenter prospective randomized therapeutic trial in advanced (stage II-IV disease, Ann Arbor classification) high-grade malignant non-Hodgkin's lymphomas (NHL, Kiel classification) a sequential combination of the COP-BLAM (5 cycles) and the IMVP-16 (2 cycles) protocols was employed. Response was first determined after 2-3 cycles. In a response-adapted manner the therapy was immediately switched to IMVP-16 if only a partial remission or no response was obtained as evidenced by the first restaging. The aim of the study is the investigation of the efficiency of this concept to induce stable remissions. In an additional randomized trial, involving all patients reaching complete remissions after chemotherapy (second restaging), the prognostic relevance of adjuvant radiotherapy as compared to therapy-free follow-up is evaluated. Eighty percent of the 191 recruited qualified patients have so far become evaluable. Complete clinical remissions were achieved in 76/148 (51%) of the patients up to the first, in 52/85 (61%) of the patients up to the second restaging. Only in a few cases did the expected toxicity of intensive polychemotherapy reach WHO grade 3-4, including nausea and diarrhea, infections, septic complications, myelotoxicity, and stomatitis. Four of the 29 deaths recorded so far occurred in complete remission due to treatment-related complications, whereas 22/29 (76%) died in progression and 3 of unrelated causes.
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PMID:[Multicenter prospective risk-adapted study on the therapy of non-Hodgkin's lymphomas of high malignancy. Use of COP-BLAM/IMVP-16 and randomized adjuvant radiotherapy--study concept and preliminary results]. 245 92

Since 1984 bone marrow from 42 children with acute lymphoblastic leukaemia, non Hodgkin's lymphoma and neuroblastoma was cryopreserved. In 5 cases (c-ALL, NHL and B type) the marrow was purged by using a cocktail of three monoclonal antibodies (VIL A1, VIB C5, VIB-E3). Up to now 13 children (ALL/10, neuroblastoma/3) were autografted (one of them after purging) after supralethal chemoradiotherapy. Except one child with early death all patients had engraftment: a level of 1.0.10(9)/l leukocytes was reached at days 10-33 (median, 19); platelet level over 60.10(9)/l at days 32-60 (median, 41). 2 children died on treatment related complications, one on infection after full haematological restitution, 2 patients alive with relapse, 8/13 alive in CCR and well.
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PMID:Cryopreservation of marrow, purging and autologous bone marrow transplantation in childhood. 248 Mar 16

We investigated the possibility that T cells observed in lymph nodes involved by B-non-Hodgkin's lymphomas (B-NHL) may have a direct role in the expression of Mu- or Gamma- heavy chain isotype by autologous malignant B cells. T cells were separated from lymph nodes involved by B-NHL cells expressing either surface IgM (19 cases) or surface IgG (4 cases) and compared to peripheral blood T lymphocytes of healthy subjects (19 cases) for their ability to promote both IgG and IgM secretion in Cowan-activated normal B lymphocytes. The mean values of IgG/IgM ratios obtained under the influence of T cells associated with malignant B cells expressing either surface IgM or surface IgG were not statistically different to that obtained with the help of control T cells (0.60 and 0.62 versus 0.47, respectively). These results do not account for the hypothesis that autologous lymph node T cells may directly affect the expression of the heavy chain isotype by malignant B-NHL cells.
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PMID:Role of autologous lymph node T cells in membrane expression of mu- or gamma-heavy chain isotype by malignant B NHL cells. 251 Oct 37

We considered the prognostic factors in high-grade non-Hodgkin's lymphomas (HG-NHL) over the past two decades. In an effort to clarify the relationship between prognostic factors and therapy, we pooled the literature reports concerning 3,480 patients into four different periods according to the mean years of the clinical trials. The most important prognostic factors discovered in period A (mean year prior to 1970) were histology, symptoms and stage. In period B (1970 through 1975), in addition to the former indicators, two new factors were pointed out: bone marrow involvement and serum lactic dehydrogenase. In period C (1976 through 1980) the significance of stage was reduced, while bulk and measures of lymph nodal and extranodal involvement (LSI, ESI) were found to be better prognostic factors. In studies related to this period the prognostic role of albumin, hemoglobin and erythrocyte sedimentation rate were also emphasized. Period D (1980 through 1985) was characterized by a decrease in the importance of the Kiel and Working Formulation (WF) classifications by virtue of the better outcome, in different reports, of HG-NHL with respect to low-grade NHL. The conclusion of our analysis is that symptoms, ESI, bulk, LDH, albumin and hemoglobin should be the most important factors used today in planning the therapy and management of patients with HG-NHL. In addition, an update of the WF is necessary.
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PMID:Relationship between prognostic factors and therapy in high-grade non-Hodgkin's lymphomas over two decades. 251 Nov 24

Cells belonging to the mononuclear phagocytic system may give rise to a wide variety of proliferative disorders. These neoplastic or reactive diseases of monocytes, histiocytes and macrophages are still poorly understood, both from a biological, clinical and pathological point of view. In 1984 a new type of non-Hodgkin's lymphomas has been described, which recently was termed large cell anaplastic lymphoma (LCAL). Since both "true" malignant histiocytosis (MH) and LCAL display some clinico-pathological similarities, we reevaluated eleven children with the primary diagnosis of MH, who entered the multicentric therapy study DAL-HX 83. On the basis of the typical morphology LCAL and not MH has been diagnosed in all cases. The Ki-1 (CD 30) positive tumor cells of LCAL can display different phenotypes, either T-lymphoid, B-lymphoid, histiocytic or neither of them (O-phenotype). Therefore we investigated the lineage specificity of tumor cells in 15 paediatric patients with LCAL. Tumor cells of histiocytic origin could be demonstrated in only one of them. In 7 patients the neoplastic cells were of T cell and in the remaining 7 cases of non T/non B cell (O) phenotypes, confirming the extreme rarity of MH in children. It is our opinion that MH and LCAL seem to be different diseases not only nosologically but also clinico-pathologically. In the multicentric therapy studies NHL-BFM 81 and NHL-BFM 83 the frequency of LCAL in childhood is compared to all other subentities at 3%.
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PMID:[Definition of malignant histiocytosis and large cell anaplastic (Ki-1) lymphoma in children]. 255 Jun 97

The mummy cell is a necrotic single cell in lympho-reticular lesion, and it is characterized by the pattern of distributing isolated in a lympho-reticular background without detachment from the surrounding cells, staining deeply or amphibic in mature both nuclei and cytoplasm and the ghost nuclei are often recognized. Totally, 95 lympho-node biopsies were reviewed, the mummy cells can be found in 21/24 of Hodgkin's disease and 18/71 of other lesions (NHL, reactive hyperplasia etc.). They are more common in nodular sclerosis (NS) and lymphocyte depletion (LD) among the four subtypes of Hodgkin's disease. It is worthwhile to note the suggestive role of mummy cells in pathological diagnosis of Hodgkin's disease.
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PMID:[The significance of mummy cell in the diagnosis of Hodgkin's disease]. 258 54

Splenectomy has a major role in the treatment of hematologic diseases. Although it is rarely curative, splenectomy removes the site of the destruction or sequestration of erythrocytes, leukocytes, and platelets. Destruction occurs in such diseases as hemolytic anemia and ITP, whereas sequestration occurs as an idiopathic disease or secondary to a host of other diseases described above. Splenectomy is also indicated for symptomatic splenomegaly associated with leukemia, lymphoma, or myeloproliferative disorders. It is palliative, increasing the comfort of the patient by removing a massive spleen. Splenectomy also serves as a staging tool in lymphoma, Hodgkin's disease, and NHL, although it is much more beneficial in Hodgkin's disease, owing simply to stage at presentation. Splenectomy can be performed safely with minimal risk in most patients, but certain diseases carry increased risks of fatality and complications. Such diseases include leukemia, lymphoma, and myeloproliferative disorders, but even in these, splenectomy may be indicated in a select group of patients. The decision to perform splenectomy should therefore be made individually in these cases. Because it can be palliative in some cases, splenectomy can play a role in patient management, even though it may not alter survival.
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PMID:Splenectomy for hematologic disease. 264 43

Using the polymerase chain reaction we examined for specific Ig kappa-L chain V region gene (V kappa gene) rearrangement in small lymphocytic non-Hodgkin's lymphomas that express Ig bearing a major kappa-L chain associated cross-reactive Id, designated 17.109. Previously, we identified the 17.109-cross-reactive Id in chronic lymphocytic leukemia as a serologic marker for expression of a highly conserved V kappa gene, designated Humkv325. Using sense-strand oligonucleotides specific for the 5'-end of this V kappa gene and antisense oligonucleotide specific for a J kappa region consensus sequence, we could amplify specifically Humkv325 when juxtaposed with J kappa through Ig gene rearrangement. This allowed us to amplify rearranged V kappa genes from DNA isolated from minute amounts of lymphoma biopsy material for molecular analyses. Our studies demonstrate that 17.109-reactive SL NHL, with or without associated CLL, rearrange, and presumably express, Humkv325 without substantial somatic diversification. Our data suggest that malignant B cells in SL NHL, in contrast to NHL of follicular center cell origin, may express immunoglobulin variable region genes with little or no somatic hypermutation.
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PMID:Ig V region gene expression in small lymphocytic lymphoma with little or no somatic hypermutation. 266 89


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