Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cancer deaths among close relatives of 19 patients with bilateral retinoblastoma were reviewed. Altogether 18 deaths of nonocular cancer were found in 5 families. These constitute 5% of the entire material of 365 relatives. The main types of cancer were stomach (6 cases), bronchial (3 cases), urinary bladder carcinoma (2 cases), as well as 2 cases of reticulosarcoma and 1 of leukemia. The retinoblastoma gene may also be associated with higher incidence of cancer in families of retinoblastoma patients.
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PMID:Excess of cancer deaths in close relatives of patients with bilateral retinoblastoma. 649 95

The true survival rates for the various forms of childhood cancer are best determined from a population-based study rather than from the results of clinical trials. Population-based survival rates have been calculated for four periods between 1956 and 1980 in Queensland. There was a significant improvement in survival for children who developed cancer after 1973 compared with those diagnosed before this date. There has however been no significant improvement in the survival rate for childhood cancer overall, or for acute lymphoblastic leukaemia since 1973. Over the 25 year period significant trends in survival rates were seen in acute lymphoblastic leukaemia, non-Hodgkin's lymphoma, Hodgkin's disease, Wilms' tumour, medulloblastoma, and retinoblastoma. No such trend was seen for acute non-lymphoblastic leukaemia, neuroblastoma, rhabdomyosarcoma, juvenile or anaplastic astrocytoma, brain stem glioma, histiocytosis X, or bone tumours. There is a need for continuing research into better methods of treatment of childhood cancer.
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PMID:Childhood cancer survival trends in Queensland 1956-80. 658 17

This case represents leukemia and retinoblastoma in two separate children of a patient with retinoblastoma. This occurrence of retinoblastoma and acute lymphoblastic leukemia in the same sibship is uncommon and emphasizes multiple occurrence of some tumors.
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PMID:Association of retinoblastoma and acute lymphoblastic leukemia in a family. 695 70

Twenty-nine children with tumours that had failed to respond to conventional therapy have been treated with AMSA. There were 16 patients with haematological malignancies in whom treatment was initiated at 25 mg/m2 for 3 days, increasing to 150 mg/m2 for 5 days. There were one complete and four partial remissions in these patients, all of whom had received at least 500 mg AMSA/m2. Thirteen children with solid tumours were treated. They received single doses of 120 mg/m2 initially, increasing to 100 mg/m2 for 5 days. No complete or partial responses occurred, but some antitumour activity was noted in neuroblastoma and retinoblastoma. Dose-related severe bone marrow toxicity occurred, but gastrointestinal and other toxicity was mild. An additional patient with T cell lymphoma, who received AMSA prior to a successful autologous bone marrow transplant, is described. AMSA is an active drug in childhood leukaemia. Further studies at the maximum tolerated dose are needed to assess enough patients with any single solid tumour type. In particular, the response of neuroblastoma warrants further study. Investigation of the use of AMSA either prior to bone marrow transplantation in leukaemia or in association with autologous marrow transplant in neuroblastoma and other solid tumours may be of value.
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PMID:Phase I and II study of AMSA in childhood tumours. 695 93

A review of the literature indicates that black children in the United States have a lower overall incidence of cancer and are less prone to leukemia and certain solid tumors, including neuroblastoma, rhabdomyosarcoma, Ewing's sarcoma, testicular tumors, liver tumors, and malignant melanoma, than are white children. Black children with acute lymphoblastic leukemia and retinoblastoma, but not with neuroblastoma, Wilms' tumor, and rhabdomyosarcoma, have poorer survival rates than white children. Socioeconomic status appears to be an important reason for the discrepant outlook, but genetic differences may also play a role. Consideration of these issues will assist in planning appropriate treatment regimens.
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PMID:Cancer in black children. 698 10

The surface fixation method has been found to be a reliable procedure for detection of antibodies of retrogenetic origin in cancer serum and also for specific immunoglobulins stimulated by antigens sinthezized in malignant cells. An insoluble extract obtained from human placentas has been used for detection of retrogenectic antibodies and with soluble substances obtained from the urine of patients it has been possible to detect what seem to be specific antibodies in retinoblastoma, Hodgkin, sarcoma and carcinoma. However with a urine extract from leukemia patients the positive reactions occur with leukemia serum from leukemia as well as with the related lymphoma and myeloma. Circulating tumor associated antigens can be detected in mixtures of an anti-fetal serum with cancer serum, but cross-reactions have been found in similar tests with pregnant women's serum.
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PMID:A simple test for detection of specific and unspecific immunological reactions in cancer. 739 35

Trisomy 12 and a deletion of chromosome 13 are the most common chromosome abnormalities in patients with B cell chronic lymphocytic leukemia (B-CLL). We determined the frequencies of these abnormalities in Japanese B-CLL patients by FISH in interphase nuclei. Specimens from 42 patients were analyzed using both DNA probes specific to the centromeric region of chromosome 12 and the retinoblastoma (RB) gene. Among 42 patients, eight had trisomy 12 and 12 had the RB gene deletion. We found aberrations of trisomy 12 and the RB gene deletion in a totally different group of patients. This suggested that the trisomy 12 and the RB gene deletion occur in different clones and the presence of which in the same patient may be rare. Furthermore, the frequency of trisomy 12 (19%) found in Japanese B-CLL was lower than that in Western countries (30-35%). On the contrary, the frequency of the RB gene deletion (28.6%) was almost the same as in European B-CLL (30-35%). These results will be helpful in understanding the leukemogenesis of B-CLL.
Leukemia 1995 Nov
PMID:Independent clones of trisomy 12 and retinoblastoma gene deletion in Japanese B cell chronic lymphocytic leukemia, detected by fluorescence in situ hybridization. 747 69

Rearrangements of the retinoblastoma (RB) gene have been reported in a few cases of myelodysplastic syndromes (MDS). In addition, low or absent expression of the RB protein is found in 20-30% of cases of acute myeloid leukaemias (AML), particularly in AML with a monocytic component (M4 or M5). We performed Southern blot analysis of the RB gene in 90 cases of MDS, including 37 cases of chronic myelomonocytic leukaemia (CMML). None of them had progressed to AML at the time of study. In 37/90 patients (including 20 CMML) Northern blot analysis, study of RB protein by immunocytochemistry on bone marrow slides, and detection of point mutations in exons 20-24 of the RB gene was also made, using single strand conformation polymorphism analysis (SSCP). No abnormal Southern profile was found in any of the 90 patients. Northern blot and immunocytochemical study of RB protein were normal in the 37 cases studied. SSCP analysis detected a point mutation in 2/37 patients tested. Direct sequencing confirmed the mutation in each case, which involved intron 21 and intron 23, respectively, and was located outside splicing sites of the neighbouring exons. These findings suggest that abnormalities of the RB gene and its expression must be very rare in MDS, and play a minor role, if any, in the pathophysiology of those disorders, at least before progression to AML.
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PMID:Inactivation of the retinoblastoma gene appears to be very uncommon in myelodysplastic syndromes. 752 20

The retinoblastoma susceptibility gene in leukemia and lymphoma has been investigated using different approaches involving either gene or protein analysis. In this study, a novel method, which evaluates the functional status of the retinoblastoma gene product by a binding assay to an in vitro-translated viral oncoprotein, has been applied to leukemic cells from acute myeloid leukemia patients. One hundred twenty-two cases were considered, and 42 of them were also analyzed by Western blot. Results obtained with the two methods were comparable, with the exception of few cases, where the retinoblastoma protein appeared detectable but unable to bind to the viral oncoprotein. The retinoblastoma protein has been found defective mostly in the M3 promyelocytic subtype.
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PMID:The retinoblastoma gene product in acute myeloid leukemia: a possible involvement in promyelocytic leukemia. 755 27

Two children presenting with sporadic unilateral retinoblastoma and exhibiting a high degree of chromosome breakage were noted to have unusual facies, microcephaly and abnormal skin pigmentation. In the first child the pattern of both spontaneous and mitomycin-C-induced chromosome breakage was characteristic of Fanconi's anaemia although the degree of breakage was extreme. She also exhibited a striking increase in X-ray-induced chromosomal damage in G0 lymphocytes as measured by dicentric formation and increase in chromatid-type aberrations. She had a number of typical clinical features, including cafe-au-lait patches and abnormalities involving the kidney; however, she demonstrated neither the hypoplasia of radius and thumb nor the typical aplastic phase of this disorder. At age 22 months the child became anaemic with trilineage myelodysplasia, which was rapidly followed by the development of acute myeloblastic leukaemia. The early onset (at age 4 months) of retinoblastoma may have been associated with the underlying genomic instability. The second child exhibited a pattern of chromosome breakage characteristic of Bloom's syndrome, in addition to a moderate increase in damage induced by mytomycin-C. She had the typical stunted growth and malar hypoplasia of Bloom's syndrome although she did not demonstrate the frequently described erythematous 'butterfly rash' Although patients with Fanconi's anaemia and Bloom's syndrome are recognised to be at an increased risk of cancer, retinoblastoma has not previously been described in patients with either condition. We suggest that underlying recessive chromosome breakage syndromes may be underdiagnosed in paediatric cancer patients, with important implications for prognosis and genetic counselling.
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PMID:Retinoblastoma in association with the chromosome breakage syndromes Fanconi's anaemia and Bloom's syndrome: clinical and cytogenetic findings. 755 65


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