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 biological characteristics and prognosis of childhood malignant lymphoma were reviewed. A national survey of 568 cases of childhood malignant lymphoma in Japan revealed that 505 cases (88.9%) were non-Hodgkin's lymphoma (NHL), and 63 cases (11.1%) were Hodgkin's lymphoma (HL). The biological characteristics of NHL in Japan were a male predominance, an average age of 7.8 years, and frequent occurrence in the head and neck (38.2%). Histopathologically, only 3.2% of NHL was nodular type and 96.8% was diffuse type (lymphoblastic 28.4%, histiocytic 19.5%, medium-size cell 18.6%, Burkitt's 14.6%, and miscellaneous 15.8%.) The characteristics of the 63 cases of HL were a male predominance, an average age of 9.3 years, 73% of primary lesions in the cervical nodes, and 46.6% of mixed cellularity type. The survival rate at 7 years estimated by the Kaplan -Meier method was 41.1% for patients with NHL, and 84.9% for patients with HL. Leukemic conversion and CNS involvement occurred in 27.9% and 22.9% of patients with NHL, respectively. The current multi-institutional treatment study for NHL conducted by the Children's Cancer and Leukemia Study Group (CCLSG) has improved the prognosis of childhood NHL to an 84.6% induction rate with initial therapy and a relapse-free survival rate of 55.6% at four years. The most important principle for the management of childhood malignant lymphoma is the recognition of the systemic nature of the disease.
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PMID:[Biological characteristics and prognosis of childhood malignant lymphoma]. 380 Apr 7

The childhood non-Hodgkin's lymphomas (NHL) are histologically, immunologically, and clinically a heterogeneous group of diseases. Recent advances in our understanding of NHL have demonstrated the similarities between childhood NHL and childhood acute lymphoblastic leukemia (ALL). Treatment strategies utilizing systemic chemotherapy and modeled after successful treatment programs for ALL have resulted in a dramatic improvement in prognosis for children with NHL, and the majority of affected children are now curable.
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PMID:Non-Hodgkin's lymphoma in children. 388 3

178 children presenting with non Hodgkin's lymphomas (NHL) were treated using the same protocol from 1973 to 1978 at the Institut Gustave-Roussy. They were classified according to stages (13 stage I, 21 stage II, 80 stage III, 50 stage IV, 6 undetermined stage), according to initial site of involvement (abdomen: 68, mediastineum 55, ORL: 32, lymph glands: 13, "others": 10) and according to histology. They were given combination chemotherapy (Vincristinee-adriamycin-cyclophosphamide-prednisone), preventive CNS therapy by cranial irradiation and intrathecal methotrexate (except in stage I patients) and maintenance therapy by cyclic multiple agents regimens including vincristin-adriamycin, vincristin-cyclophosphamide and cytarabine-asparaginase. Radiation therapy was carried out up to 1977 in stage I and II patients only. Combination chemotherapy improved global survival of pediatric NHL (40% at 4 years, all staged added); however improvements remain to be done in patients with widespread disease.
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PMID:[Results of treatment of 178 pediatric non Hodgkin's malignant lymphomas between 1973 and 1978 (author's transl)]. 725 19

Twentynine children with non-Hodgkin's lymphomas (NHL) were treated between 1974 and 1977 with a protocol based on those used for childhood ALL. 76% of patients had advanced disease by Ann Arbor criteria. All tumours had Rappaport's diffuse histology. 19 patients (65%) achieved complete remission, 14 (65%) remained alive and disease free beyond 42 months from diagnosis. 10 patients failed to enter complete remission, of whom all died. 7 patients relapsed; 5 died, 2 remain disease free and off treatment at 19 and 29 months. Comparison with a historic group of 20 consecutively treated children shows improved survival (P less than 0.01). 18 controls died. Histology was reviewed using the Kiel classification and staging according to Murphy's criteria. These are compared with the methods used initially. The improved outlook for children with NHL using intensive multiple drug regimes and cranial prophylaxis is confirmed. In staging childhood NHL, Murphy's criteria, which take into account the natural history of the disease, have greater prognosis value. Histology and pattern of outcome of the disease suggest basic differences between primary abdominal and primary mediastinal and nodal disease. This is now being confirmed with immunological typing and will be reflected in the development of future protocols.
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PMID:The long-term outlook for children treated for non-Hodgkin lymphomas. A report of the Children's Solid Tumour Group. 732 96

The types of childhood non-Hodgkin lymphoma (NHL) differ considerably from Hodgkin lymphoma and NHL seen in adults, both pathologically and clinically. Essential to understanding these differences is a knowledge of the three major histologic subtypes (undifferentiated, lymphoblastic, and large cell) that account for the vast majority of cases of pediatric NHL. Each of these subtypes has typical imaging and clinical features. The most common subtype, undifferentiated NHL, usually shows intraabdominal disease. Lymphoblastic tumors most frequently manifest as a mediastinal mass, perhaps with respiratory or circulatory compromise. Large cell tumors show heterogeneous clinical and imaging features but tend to spare the anterior mediastinum. Knowledge of the appropriate imaging modality to be used in evaluation of these tumors is also important. Computed tomography (CT) is the primary imaging modality for staging childhood NHL. Magnetic resonance imaging is best for examination of the central nervous system and bone involvement. Ultrasonography may be useful as a complementary study to abdominal CT; gallium scintigraphy also plays an adjunctive role to CT. Familiarity with typical and atypical patterns of tumoral behaviors and optimal imaging methods aid in the diagnosis and appropriate follow-up of these tumors.
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PMID:Imaging of childhood non-Hodgkin lymphoma: assessment by histologic subtype. 812 42

To evaluate the role of serum CA 125 levels for the diagnosis, follow-up, and prognosis in childhood non-Hodgkin's lymphomas (NHL), 44 children (35 newly diagnosed patients and 9 patients with relapsed or progressive disease, median age 6.5 years, M/F ratio 2.1) with NHL were included in this study. CA 125 levels in serum and/or ascites and effusions were measured by radioimmunoassay. The upper limit of normal was 35 IU/mL. The correlation of CA 125 levels with tumoral location and treatment results were investigated. A total of 27 (61.4%) patients had increased and 17 patients had normal serum CA 125 levels. Fifteen patients with increased CA 125 levels had malignant ascites, pleural effusion, or both, although none with normal CA 125 levels had any serous membrane involvement. The mean CA 125 levels were 72.5 IU/mL in patients with no serosal involvement (ascites or effusion) and, 144.0, 273.7, 324.3 IU/mL in patients with pleural effusion, ascites, and ascites + effusion, respectively. The mean CA 125 levels in ascites and pleural effusion were 202.1 and 156.2 IU/mL, respectively, and were similar to the corresponding serum levels. The increased CA 125 levels returned to normal in 18 patients whose diseases were in remission during the follow-up. One-year survival rate for newly diagnosed patients was 80.2 and 87.5% in patients with increased and normal serum CA 125 levels, respectively. In conclusion, CA 125 levels were significantly higher in patients with serous membrane involvement and there was a correlation between treatment response and marker levels. Little is known about the expression of CA 125 in NHLs. This is the first report suggesting that serum CA 125 levels can be a useful marker for the follow-up of childhood NHL. CA 125 seems to be a promising tumor marker in the assessment of prognosis and therapeutic response in non-Hodgkin's lymphomas.
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PMID:Serum CA 125 levels in children with non-Hodgkin's lymphoma. 1040 67

While many pediatric malignancies are seen predominantly in pre-school children, many cases of childhood non-Hodgkin's lymphoma and most cases of Hodgkin's disease and bone tumors are seen in the older child and adolescent. This review focuses on current knowledge concerning the epidemiology, histopathology, molecular biology, clinical presentation, diagnosis, staging, treatment, and prognosis for older children and adolescents diagnosed with lymphoma or either of the two commonly seen childhood bone tumors, namely osteosarcoma and Ewing's sarcoma. Survival figures for all of these childhood malignancies have increased markedly in the past two decades. We now have the relatively new experience of having an increasingly large population of childhood cancer survivors to study and, unfortunately, are beginning to see the long-term consequences of these more successful treatments. This review concludes with an overview of the potential late effects of cancer therapy, effects that may first be detected by the primary care physician caring for the adolescent who is a cancer survivor.
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PMID:Lymphomas and bone tumors: clinical presentation, management, and potential late effects of current treatment strategies. 1061 39

Non-Hodgkin's lymphomas (NHL) in children and adolescents represent about 10% of childhood cancers. Although the types of NHL commonly seen in this population are relatively limited to lymphoblastic lymphomas, Burkitt's and Burkitt-like lymphomas, and large cell lymphomas, correct diagnosis and classification are essential for optimal therapy. Careful handling of pathologic specimens, along with collection of proper materials for ancillary studies such as immunophenotyping, cytogenetics, or molecular studies, will aid the pathologist in reaching a correct diagnosis. Specific morphologic, immunophenotypic, and genetic features of the commonly seen types of pediatric NHL are described.
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PMID:Work-up and diagnosis of pediatric non-Hodgkin's lymphomas. 1089 Feb 53

Despite its clinical and histological heterogeneity, anaplastic large cell lymphoma (ALCL) is now a well-recognized clinicopathological entity accounting for 2% of all adult non-Hodgkin's lymphomas (NHL) and about 13% of pediatric NHL. Immunophenotypically, ALCL are of T cell (predominantly) or Null cell type; by definition, cases expressing B cell antigens are officially not included in this entity. The translocation (2;5)(p23;q35) is a recurring abnormality in ALCL; 46% of the ALCL patients bear this signature translocation. This translocation creates a fusion gene composed of nucleophosmin (NPM) and a novel receptor tyrosine kinase gene, named anaplastic lymphoma kinase (ALK). The NPM-ALK chimeric gene encodes a constitutively activated tyrosine kinase that has been shown to be a potent oncogene. The exact pathogenetic mechanisms leading to lymphomagenesis remain elusive; however, the synopsis of evidence obtained to date provides an outline of likely scenarios. Several t(2;5) variants have been described; in some instances, the breakpoints have been cloned and the genes forming a new fusion gene with ALK have been identified: ATIC-ALK, TFG-ALK and TPM3-ALK. Cloning the translocation breakpoint and identifying the ALK and NPM genes provided tools for screening material from patients with ALCL using various approaches at the chromosome, DNA, RNA, or protein level: positive signals in the reverse transcriptase-polymerase chain reaction (RT-PCR) and the immunostaining with anti-ALK monoclonal antibodies (McAb) serve as the most convenient tests for detection of the t(2;5) NPM-ALK since the fusion gene and ALK protein expression do not occur in normal or reactive lymphoid tissue. The wide range of NPM-ALK positivity reported in different series appears to be dependent on the inclusion and selection criteria of the ALCL cases studied. Overall, however, 43% of ALCL cases were NPM-ALK+ (83% of pediatric ALCL vs 31% of adult ALCL). Occasional non-ALCL B cell lymphomas (4%) with diffuse large cell and immunoblastic histology and Hodgkin's disease cases (3%) were NPM-ALK-, but these data are questionable. The aggregate results indicate that, in contrast to primary nodal (systemic) ALCL, the t(2;5) may be present in only 10-20% of primary cutaneous ALCL and rarely, if at all, in lymphomatoid papulosis, a potential precursor lesion; however, these 10-20% positive cases were not confirmed by anti-ALK McAb immunostaining and may represent an overestimate. Positivity for NPM-ALK is associated to various degrees with the following parameters: 44% and 45% of ALCL cases with T cell and Null cell immunophenotype, respectively, are positive, whereas only 8% of cases with a B cell immunoprofile are positive; the mean age of positive patients is significantly younger than that of negative patients; positive cases carry a better overall prognosis (but not in all studies). Recently, the homogenous category of ALK lymphoma ('ALKoma') has emerged as a distinct pathological entity within the heterogenous group of ALCL. The fact that patients with ALK lymphomas experience significantly better overall survival than ALK- ALCL demonstrates further that analysis of ALK expression has important prognostic implications. The term ALK lymphoma signifies a switch in the use of the diagnostic criteria: cases are selected on the basis of a genetic abnormality (the ALK rearrangement), instead of the review of morphological or immunophenotypical features which are clearly more prone to disagreement and controversy. Since its initial description in 1985 ALCL has become one of the best characterized lymphoma entities.
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PMID:Pathobiology of NPM-ALK and variant fusion genes in anaplastic large cell lymphoma and other lymphomas. 1099 99

The aim of this study was to investigate the late effects of treatment for childhood non-Hodgkin's lymphomas (NHL) on oral health and dental development. Thirty-six long-term survivors that had been treated with chemotherapy of childhood NHL were included in this study and 36 volunteers with similar age and sex distribution served as controls. Both groups underwent a complete orodental examination for decayed, missing and filled teeth and surfaces, gingival and periodontal health according to the Loe-Silness Gingival Index and Sillnes-Loe Plaque Index, enamel defects and discolorations, root malformations, eruption status, agenesis, premature apexifications and microdontia. The severity of these disturbances related to age at the time of NHL diagnosis were also evaluated by creating two groups as < 5 yr and > 5 yr. Although none of the parameters altered with age, patients had significantly higher plaque index, more enamel discolorations and root malformations than did the controls. The results show that long-term survivors of NHL patients exhibit some orodental disturbances that may be attributed to the chemotherapy regimens.
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PMID:Long-term effects of chemotherapy on orodental structures in children with non-Hodgkin's lymphoma. 1487 Nov 87


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