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)

Intradermal skin test antigen (Varidase, Mumps, Candidine) and contact sensitization with 2,4-dinitrochlorobenzene (DNCB) were employed in 35 previously untreated children with neuroblastoma and malignant lymphoma, to evaluate delayed cutaneous hypersensitivity. Cutaneous responses were correlated to stage of disease and survival. The limited number of patients prevents any definitive conclusion. It is apparent, however, that in childhood non-Hodgkin's lymphoma anergy to DNCB is correlated to advanced stages with rapid progressive disease. In neuroblastoma and Hodgkin's disease patients skin test sensitization seems to be of questionable clinical value.
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PMID:Skin tests in childhood lymphoma and neuroblastoma. 102 49

We report here cytogenetic studies of a series of 23 childhood non-Hodgkin's lymphomas (NHL), a group that has previously not been subjected to detailed cytogenetic analysis. Combining our results with data from 25 tumours in the published literature, we have performed the first cytogenetic analysis of a large series of childhood NHL. Our results show that the cytogenetic changes encountered in NHL of children are distinct and may be different from those seen in NHL of adults reflecting the previously recognized differences in histological presentation and clinical behaviour of the two entities. Thus, the most frequently occurring translocation in B-cell lesions in children was t(8;14)(q24;1q32). Other translocations frequently seen in adults such as t(14;18)(q32;q21),t(11;14)(q13;q32) and t(3;22)(q27;q11) were either rare or so far not seen in children, although reciprocal translocations appeared to be generally prevalent in childhood NHL. Combining our data with those in the published literature, we have identified two new recurring translocations [t(1;17)(p36;q21) and t(1;14)(p36;q22)], and a recurring duplication [dup(11)(q13;q23)] in this group of lymphomas. In addition, our literature survey identified a third recurring translocation [t(5;14)(q23;q32)] which was previously reported in two cases of childhood NHL. Our analysis also showed differences in the types of nonrandom translocations between childhood NHL and acute lymphoblastic leukaemia (ALL) in children suggesting that biologically these entities are different from one another. This study thus uncovers patterns of chromosome change associated with childhood lymphoma thus providing new opportunities for investigation of their clinical significance by correlation analysis and biological significance by molecular analysis.
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PMID:Distinct patterns of chromosome abnormalities characterize childhood non-Hodgkin's lymphoma. 153 6

Cytogenetic studies were performed successfully on 24 patients with non-Hodgkin lymphoma (NHL) who were younger than 15 years of age. Of these, 22 patients (92%) had abnormal clones. With respect to histologic findings, 3 (25%) of the 12 patients with lymphoblastic lymphoma had 14q11 translocations and 2 (17%) had t(9;17) (q34;q23). Four (80%) of the five patients with small non-cleaved cell lymphoma had t(8;14)(q24;q32). With respect to immunologic findings, four (44%) of the nine patients with T-cell lymphoma had abnormalities consisting of 14q11 and 7q36 translocations, in which the T-cell receptor genes resided. Three (33%) of the patients with T-cell lymphoma had t(9;17)(q34;q23). However, three (43%) of the seven patients with B-cell lymphoma had t(8;14) (q23;q32), and two (29%) of the patients with B-cell lymphoma had an extra i(11q) chromosome with a resultant 11q tetrasomy. Non-T-cell non-B-cell lymphomas, which occurred in 21% of all patients, showed various chromosomal abnormalities. This study demonstrated that, in childhood NHL, karyotype correlates closely with immunophenotype, clinical features, and histologic findings.
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PMID:Cytogenetic characteristics of childhood non-Hodgkin lymphoma. 162 87

Uncommon histologies were identified in 36 of 1336 cases (2.7%) of newly diagnosed childhood non-Hodgkin lymphoma (NHL). Seventeen cases were classified as follicular (six cases as mixed small and large cell, nine as large cell, and two as small non-cleaved cell) and 19 cases as diffuse (18 cases as mixed small and large cell, and one as small cell lymphocytic). The follicular pattern group included a preponderance of male patients; the median age at diagnosis was 11.7 years. These children presented primarily with low-stage disease involving lymph nodes or tonsils. All patients except one achieved complete remission and remain disease-free for 11 months to 18.8 years (actuarial 5-year event-free survival, 94%). The group with diffuse histologies was similar in sex ratio, age at diagnosis (median = 12.1 years), and nodal involvement, but tended to have more advanced-stage disease. Moreover, only 14 of 19 (74%) children with diffuse intermediate-grade histologies are alive in continuous complete remission (actuarial 5-year event-free survival, 70%). These results suggest that follicular pattern childhood NHL has an excellent prognosis, whereas cases with diffuse intermediate-grade histology are prognostically similar to those with diffuse high-grade histologies.
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PMID:Childhood malignant non-Hodgkin lymphomas of uncommon histology. 164 Jul 26

The authors retrospectively reviewed the clinicopathologic and immunologic features of 65 consecutive cases of childhood lymphoma reported between 1980 and 1989. Southern blot hybridization was also performed in 23 cases to study their association with Epstein-Barr virus (EBV) and human T-cell leukemia virus type 1 (HTLV-1). The 65 cases included 56 non-Hodgkin's lymphoma (NHL) (86%) and 9 Hodgkin's disease (HD) (14%). The NHL could be classified into the following groups: Group I, small noncleaved cell lymphoma (20 cases); Group II, lymphoblastic lymphoma (17 cases); Group III, large cell lymphoma (17 cases); and miscellaneous (2 cases). There was no follicular lymphoma case. Immunohistochemical study on paraffin sections and/or frozen specimens in 47 cases of NHL showed that all the Group I cases belonged to B-cell neoplasm (17 of 17 cases); most of the Group II cases belonged to T-cell neoplasm (9 of 14 cases); and most of the Group III cases were peripheral T-cell lymphoma (PTL) (8 of 16 cases), including 2 cases of Ki-1 lymphoma. The majority of childhood NHL belonged to high-grade malignancy with an aggressive clinical course (median survival time, 8 months). The EBV DNA could be detected from the tumor tissues in 4 of 6 PTL, but in none of the remaining 19 cases of NHL including 6 Burkitt's type lymphomas. HTLV-1 proviral genome was not detected in all specimens examined. The authors concluded that the distribution pattern and clinicopathologic feature of childhood lymphoma in Taiwan are comparable to that in Japan and western countries. The frequent association of EBV with aggressive PTL was unique and deserves additional investigation.
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PMID:A pathologic study of childhood lymphoma in Taiwan with special reference to peripheral T-cell lymphoma and the association with Epstein-Barr viral infection. 165 30

The relative frequencies of the various histopathologic types of lymphomas are generally similar among Asian countries. Hodgkin's disease and follicular lymphomas are relatively rare in Asia. Among NHL, the Asians have a higher rate of aggressive NHL, as compared with the NCI data. Immunologic analysis revealed that PTCL is common in Asia. The relative frequency of PTCL is comparable among Chinese in Taiwan, the east coast of China, and Hong Kong, as well as in adult T-cell leukemia/lymphoma (ATLL) nonendemic areas in Japan. The increased rate of T-cell lymphomas in Asia is attributed to the low incidence of follicular lymphomas. The similar patterns of distribution in histopathologic and immunologic subtypes of NHL in Asia suggest that a common ethnic or geographic factor exists. To elucidate it, further detailed epidemiologic studies are needed. Primary extranodal NHL is slightly more prevalent in Asia than in the United States; the most frequent primary site is Waldeyer's ring in Japanese patients and the GI tract in Chinese patients. Primary small intestinal lymphoma in Asia showed the pattern of the Western type. Primary cutaneous lymphomas are rare in Asia. The clinical features of PTCL in Asia are comparable with those described in the United States, except for a predilection for the nasal/paranasal region. In Asia, outside Japan, ATLL has been reported only in Taiwan. The seroepidemiologic survey of carriers of ATLL showed the rate of seropositivity for HTLV-I in Taiwan was similar to that in nonendemic areas in Japan. The clinicopathologic features of ATLL in Taiwan and Japan are essentially identical. In children in Japan and Taiwan, Hodgkin's disease is much less frequent than in the West. However, the relative frequencies of the histopathologic and immunologic subtypes of childhood NHL in Japan and Taiwan do not differ significantly from those of the West. Although Burkitt's lymphoma in Japan and Taiwan is of nonendemic type, in India it may comprise both endemic and nonendemic types in almost equal number.
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PMID:Non-Hodgkin's lymphomas in Asia. 193 64

Non-Hodgkin's lymphomas (NHL) are classified as low or high grade malignant lymphomas of either B or T cell origin. Cells of low grade malignant NHL (LG-NHL) of B cell type represent either follicular or postfollicular, cells of LG-NHL of T cell type postthymic maturation stages in the lymphoid differentiation pathway. LG-NHL often fail to undergo long-term complete remission or cure in adults, no matter what type of conventional therapy is applied. In three pediatric therapy studies frequency and probability of continuous complete remission (pCCR) were studied for LG-NHL and peripheral pleomorphic T-cell lymphomas (PTCL). Of 432 evaluated patients only six children (1.4%) were qualified as LG-NHL. LG-NHL of T cell type were diagnosed in three children (one T-zone lymphoma, two PTCL of small cell type), LG-NHL of B cell type in another three cases. Two children presented with nodular type of centroblastic/centrocytic lymphoma (CB/CC), one with lymphoplasmocytoid type of immunocytoma (IC). In addition to the two patients with low grade malignant PTCL, there have been also four children with high grade malignant PTCL. The analysis showed that there was not significant difference for event free survival of children with LG-NHL and HG-NHL, respectively (pCCR: 0.63 and 0.82, p = 0.29). In contrast, when comparing high and low grade PTCL versus all other types of childhood NHL (non-PTCL), a significant difference seems to exist (pCCR for PTCL: 0.44, for non-PTCL: 0.83; p = 0.02). However, for further concisive conclusions more patients are needed for evaluation.
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PMID:Low grade malignant non-Hodgkin's lymphomas and peripheral pleomorphic T-cell lymphomas in childhood--a BFM study group report. 220 39

Between 1962 and 1986, a total of 338 consecutive newly diagnosed children and adolescents with non-Hodgkin's lymphomas (NHLs) were evaluated and treated at St Jude Children's Research Hospital (SJCRH). Median follow-up is 6.6 years (range, 1.8 to 23 years). The patients ranged in age from 7 months to 21 years (median, 10 years), and 71% were males. All cases were staged (I to IV) by a clinical staging system. Eighteen percent were stage I, 21% stage II, 43% stage III, and 18% stage IV. Cases frankly leukemic at diagnosis (ie, greater than 25% marrow blasts) were excluded from the analysis. Pathologic material from all cases was reviewed and classified according to the Working Formulation. The histologic distribution of cases was as follows: 38.8% diffuse small non-cleaved cell (undifferentiated, Burkitt's and non-Burkitt's); 26.3% diffuse large-cell, mainly immunoblastic; 28.1% lymphoblastic; and 6.8% other. Treatment policy evolved over time to a stage- and histology-specific strategy for treatment assignment, and overall results significantly improved by era from 37% (+/- 5%) 2-year event-free survival (EFS) for patients treated before 1975 to 77% (+/- 4%) since 1978. By univariate and multivariate Cox regression analyses, the era of treatment (hence, the protocol-specific treatment itself), the stage, and the log of the initial serum lactic dehydrogenase (LDH) emerged as the most powerful prognostic indicators, while histology per se was not significantly related to outcome. For the 154 patients treated since 1978, the 2-year EFS by stage was 97% (+/- 3%) for stage I, 86% (+/- 6%) for stage II, 73% (+/- 6%) for stage III, and 47% (+/- 11%) for stage IV (P less than .0001). Compared with our previous experience, we conclude that the cure rate of childhood NHL has doubled in the last decade with modern management.
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PMID:Non-Hodgkin's lymphomas of childhood: an analysis of the histology, staging, and response to treatment of 338 cases at a single institution. 278 41

Childhood non-Hodgkin's lymphomas (NHL) are a heterogeneous group. Biological studies have shown that most mediastinal NHL are lymphoblastic lymphomas which are T-cell tumors. In contrast, most NHL occurring in the gastrointestinal tract, abdominal cavity and Waldeyer's ring are diffuse undifferentiated small non-cleaved lymphomas, mainly Burkitt's or Burkitt-like lymphomas. Different multi-drug combinations have been proposed over the last 20 years. The LSA2-L2 regimen or other protocols, based on acute lymphoblastic leukemia results, have improved the prognosis in T-cell NHL. In extended B-cell NHL, aggressive short term multi-drug chemotherapy given in pulses gives a cure rate above 60%. The use of surgery and radiotherapy in the treatment of childhood NHL has decreased with the realisation that most children can be cured by chemotherapy alone.
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PMID:Childhood non-Hodgkin's lymphomas: clinical and therapeutic aspects. 319 Dec 4

The spectrum of non-Hodgkin's lymphomas (NHL) that occurs in children differs markedly from that in adults. This is probably a consequence of differences in the proportions of precursor and mature lymphoid cells in the immune systems of children and adults, and the greater emphasis on the development of an immunologic repertoire in the child. Childhood NHL can be classified into three main types based on histology, all of them diffuse: lymphoblastic, small noncleaved cell, and large cell. The majority of lymphoblastic lymphomas are of immature T cell (thymocyte) origin, although a few have a B cell precursor phenotype. All express the enzyme terminal transferase. Small noncleaved lymphomas express B cell characteristics, as do the majority do the majority of large cell lymphomas, although a small proportion of the latter express T cell characteristics. Very few are of true histiocytic origin. Little is known of the epidemiology of lymphoblastic and large cell lymphomas. However, using histology as a diagnostic criterion, both occur throughout the world and occur primarily, as do all childhood NHL, in the first two decades of life. There appear to be at least two types of small noncleaved cell lymphomas, both of which are associated with specific chromosomal translocations. An endemic form occurs at high frequency in equatorial Africa, and a sporadic form occurs at low frequency throughout the world. The endemic tumor is associated with the Epstein-Barr virus, it has a high incidence of jaw tumors, and has a breakpoint on chromosome 8 that is usually some distance upstream of the c-myc oncogene. The sporadic tumor is only occasionally associated with EBV, it often involves the bone marrow, particularly at relapse, and has a breakpoint on chromosome 8 that is usually very close to or within the c-myc oncogene. Childhood NHL is rarely truly localized, and treatment regimens are always based on chemotherapy. There is no evidence that radiation is beneficial when modern combination drug regimens are employed as the primary therapeutic modality. Prophylactic treatment to the central nervous system is recommended in nearly all patients, and intrathecal drugs, usually supplemented by some form of high-dose or intermediate-dose methotrexate, appear to represent adequate prophylaxis to the CNS. The most effective regimens result in cure in almost all patients who have limited overt disease, and in a high proportion (50 to 75 per cent) of patients with extensive disease, although patients with bone marrow involvement do poorly with most regimens.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Malignant non-Hodgkin's lymphomas in children. 332 74


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