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Biomedical subjects

T Philip

Publications and source records attributed to T Philip.

At least 325 records · Page 18Linked to original sources

Catalase determination in various etiologic forms of Wilms' tumor and gonadoblastoma.

We have previously mapped the gene coding for catalase to 11p13 by gene dosage analysis. Deletion of this chromosomal region causes aniridia, mental retardation, and predisposition to Wilms' tumor (WT). In the present study, 22 patients with various etiologic forms of WT and/or aniridia were investigated. The catalase (CAT) level and karyotype were examined in order to determine the linkage and the gene ordering on chromosome number 11 of the different loci involved. The CAT concentration was normal in the 19 cases without detectable chromosomal abnormalities.

Adolescent↗

Intensive cytoreductive regimen and autologous bone marrow transplantation in leukemia. Present status and the future. A review.

High-dose cytoreductive treatment followed by ABMT represents a new approach to the treatment of acute leukemia as an alternative when leukemic patients do not have HLA-identical donors. ABMT protocol seems to be a valuable treatment for AML if it is immediately employed after the remission obtained to consolidate the remission. For ALL adult patients, and so for poor prognosis ALL in children, intensive therapy with ABMT represents a new approach when conventional therapy has failed or failed to consolidate the remission. The results of ABMT in CML in the literature have been disappointing; the bone marrow could be collected during the course of the first chronic phase after hydroxyurea therapy or other treatment programs. In leukemia the ABMT approach will be more credible if the protocol includes in vitro immunologic or pharmacologic means to eliminate residual leukemic cells.

Acute Disease↗

Massive BACT chemotherapy with autologous bone marrow transplantation in 17 cases of non-Hodgkin's malignant lymphoma with a very bad prognosis.

A group of 12 children and 5 adults, all with diffuse non-Hodgkin's malignant lymphoma (NHML), received massive chemotherapy regimens. The stages of the disease were as follows: 7 patients were in second complete remission; 6 in a progressive phase of the disease; and 4 in first complete remission which occurred late in the course of the disease. All patients received BACT (BCNU+aracytine+cyclophosphamide+thioguanine) or TACC (idem with CCNU) at different dose levels: 6/17 received 10 Gy total-body irradiation (TBI) after BACT treatment; 16/17 received autologous bone marrow transplantation (ABMT) previously stored in liquid nitrogen to combat the medullary effects of chemotherapy. Direct therapy-related deaths occurred in 4/17 patients (1 Aspergillus endocarditis; 1 Moskowitz syndrome; 1 veno-occlusive disease of the liver; and 1 Escherichia coli pneumopathy) and 6/17 patients relapsed between days 25 and 70 of treatment. Seven out of these 17 patients are still alive NED 102-900 days (mean, 475 days) after the beginning of therapy without receiving maintenance treatment. Massive chemotherapy could thus be the best treatment for NHML in relapse, but the high percentage of early therapy-related deaths is a strong limiting factor for patients before relapse.

Adolescent↗

[Varicella-zoster virus infections in the immunosuppressed child. Treatment with Acyclovir and controls].

Sixty minutes intravenous infusions of Acyclovir were given at 5 to 10 mg/kg 3 times daily for 6 to 11 days in 20 immunodeficient children with varicella (9 cases) and herpes zoster (11 cases) in a controlled open study. Twelve patients had a life-threatening illness. All patients who received therapy before the first 4 days had a more rapid cessation of new vesicles formation and more rapid scarring. Fourteen controlled children had accelerated clearance of viral antigens from vesicles. In 19 cases, virus was no longer isolated after the 3rd day. Eighteen patients recovered within 6 to 10 days. No relapse of varicella zoster virus infections had been observed 1 to 18 months after the end of treatment. Two children with varicellous interstitial pneumonia died 8 and 32 days after the end of treatment. In 3 cases, zoster pains reappeared 8 days after Acyclovir therapy was stopped. The drug was well-tolerated, but control of renal functions is necessary.

Acyclovir↗

[Chromosomal translocation (11; 22) in cell lines of Ewing's sarcoma].

Chromosome studies were performed on 5 Ewing sarcoma cell lines. An identical reciprocal translocation t(11; 22) (q24; q12) was found in 4 cell lines established from 3 different tumors. These results, associated with those obtained at the same time and independently from fresh tumor cells, suggest that the translocation t(11; 22)(q24; q12) may be a chromosomal marker characteristic of Ewing sarcoma cells. This translocation involves the chromosome 22 on which the H-c-sis oncogene has been located; it could be used as a new tool for exploring the role of genetic transposition in the malignant cell transformation.

Adult↗

Burkitt-type lymphoma in France among non-Hodgkin malignant lymphomas in Caucasian children.

In a retrospective analysis of 87 cases of Caucasian childhood non-Hodgkin malignant lymphoma (NHML) from Lyon, France, all the case were diffuse lymphomas, but 47 were diagnosed as monomorphic small non-cleaved NHML, pathologically indistinguishable from Burkitt's lymphoma (BL). BL could then be the most frequent childhood lymphoma in France. This homogeneous series allows better definition of the characteristics of BL within NHML. Age distribution is similar to that of endemic BL, with a sex ratio of 3.7/1. Abdominal masses are initially present in 68% of the cases, whereas jaw is involved in only 4%. The disease is characterized by its overwhelming evolution in the absence of therapy. However, complete remission (CR) is usually obtained after the first chemtherapy regimen. Most relapses occur at 3-8 months. Death could be related to cerebrospinal fluid (CSF) involvement, local recurrence or secondary marrow involvement. Ninety per cent of the patients alive with no evidence of disease (NED) 8 months after CR can be considered as definitely cured. Our study on Caucasian children with NHML indicates that, from histological and clinical criteria, nearly half the cases are very similar to African BL. Even though EBV rarely associated with our cases, BL could be a worldwide lymphoma.

Abdomen↗

EBV-positive Burkitt's lymphoma from Algeria, with a three-way rearrangement involving chromosomes 2, 8 and 9.

Burkitt's lymphoma was diagnosed in male Algerian child presenting with abdominal and jaw tumours. Viral studies revealed that this non-endemic case was associated with the Epstein-Barr virus. Cytogenetic examination of tumour cells showed a complex three-way rearrangement involving chromosomes 2, 8 and 9. Our report indicates that, in Burkitt's lymphoma, not only simple but also complex translocation involving chromosome 8 may arise. This finding in a North African case emphasizes the need for more detailed study of the clinical, virological and cytogenetic features of Burkitt's lymphoma throughout the world.

Burkitt Lymphoma↗

[Autologous haematopoietic stem cell transplantation. Preliminary results of a regional multicentric study (author's transl)].

Autologous bone marrow transplantation represents a new approach to the treatment of malignant diseases when conventional therapy has failed. For this reason, the authors have collected bone marrow from 46 patients, including 24 with acute leukaemia, 7 with chronic myeloid leukaemia, 10 with lymphosarcoma and 5 with solid tumours. The mean of total cryopreserved CFU-c was 8.5 X 10(6) (range: 0.2-25). Ten cases of autologous bone marrow transplantation are reported. Seven patients had been prepared with high dosage chemotherapy alone (TACC) and three with chemotherapy combined with total body irradiation. Haematopoiesis restarted within 9 to 15 days in 5 patients and within 22 to 34 days in the other 5. Complete remission was obtained in all 5 patients with acute myeloid leukaemia grafted during their first relapse, the longest remission up to now being 390 days. One patient with chronic granulocytic leukaemia is still in second chronic phase after 360 days. Stem cells were transplanted early in the course of a T-lymphosarcoma, during complete remission; maintenance chemotherapy was withdrawn, and the chances of success of this treatment alone are being evaluated. The kinetics of blood and bone marrow CFU-c populations after transplantation were studied in 4 cases and were found to correlate closely with haematopoietic recovery following ablative bone marrow therapy. Stem cell transplantation can only be justified in acute leukaemia if it is carried out immediately after complete remission to consolidate the results and, hopefully, to prolong the remission.

Acute Disease↗

beta+ -Thalassemia intermedia. Genetic and biochemical study of a family including 3 cases.

3 cases of thalassemia intermedia have been found in the same family. The parents are not consanguineous but both come from the same town of Calabria (Italia). The mother is a heterozygote for beta-thalassemia, as well as the father whose globin chain synthesis is nevertheless balanced, thus suggesting an association with alpha-thalassemia. This hypothesis is confirmed by the fact that one of the offspring shows the typical characteristics of alpha-thalassemia heterozygosity. The 3 subjects with thalassemia intermedia are synthesizing the beta-globin chain in a proportion higher than that expected from the level of Hb A in peripheral blood. In 2 of them, the globin chain biosynthetic ratio measured in the blood reticulocytes is not significantly different from that usually observed in thalassemia major of either the beta o or beta+ type. In the third subject the globin chain synthesis is slightly less unbalanced probably because an alpha-thalassemia is also present. This suggests that factors other than a lesser imbalance in globin chain synthesis are involved in the occurrence of thalassemia intermedia. One of these factors could be a better survival of cells richer in Hb F than in Hb A, since these cells must have a lesser excess of alpha-chains.

Adolescent↗