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Biomedical subjects

D Frappaz

Publications and source records attributed to D Frappaz.

At least 145 records · Page 8Linked to original sources
PubMed1988

Collagen biosynthesis in a case of infantile myofibromatosis.

Cells from a skin nodule from a patient with a recurrent form of familial myofibromatosis were cultivated in vitro. A metabolic study showed that these cells behaved like fibroblasts with collagen synthesis, a normal percentage of type III collagen, hydroxylation rate and the ability to contract a collagen gel. The main disturbances were the decreased synthesis and increased cell multiplication rate after a lag phase. This behavior was compared with that of a fibroblast culture from normal skin.

Collagen↗
PubMed1987

Effective multiagent chemotherapy in children with advanced B-cell lymphoma: who remains the high risk patient?

Between 1981 and 1985, 50 patients, mainly children and adolescents, with advanced B-cell lymphoma were entered on a protocol comprising eight drugs: cyclophosphamide, vincristine, prednisolone, high dose methotrexate, adriamycin, BCNU, cytosine arabinoside and thioguanine. Treatment to the central nervous system consisted of intrathecal methotrexate and cytosine-arabinoside in association with high dose methotrexate without irradiation. Data was collected prospectively with regard to response rate, treatment related complications and survival. Histology was reviewed in all referred cases and in 21 there was supportive evidence from immunological and cytogenetic studies. The overall complete response rate was 86%: 31/36 stage III and 12/14 stage IV. There were four treatment related deaths. The overall disease-free survival is 75% with a median follow up of 32 months. In the group of stage IV patients 5/7 with only marrow involvement, 2/4 with isolated CNS involvement and 1/3 with combined CNS and marrow infiltration survive. All the patients with CNS involvement at presentation underwent consolidation treatment with high dose chemotherapy and bone marrow transplant. These results demonstrate the very high curability of B-cell lymphoma using intensive multiagent therapy even with advanced abdominal disease. Bone marrow infiltration does not appear to be an adverse prognostic factor in isolation from bulk disease or CNS involvement. There remain, however, two groups of patients in whom further intensification of therapy is indicated, namely, those with initial CNS involvement, especially in combination with marrow infiltration, and those with extensive multiorgan involvement at presentation who fail to achieve remission with initial therapy. For the other patients, the large majority, a reduction in the intensity and duration of therapy is currently under study.

Adolescent↗
PubMed1987

Sequential cisplatin/VM-26 and vincristine/cyclophosphamide/doxorubicin in metastatic neuroblastoma: an effective alternating non-cross-resistant regimen?

We report the results of a French multicentric pilot study of remission induction therapy in metastatic neuroblastoma. Thirty-five successive unselected patients entered the study over 1 year and were treated by alternating sequences of cisplatin/VM-26 (PE) and vincristine/cyclophosphamide/doxorubicin (CADO). Three courses of each sequence were delivered. Disease reevaluation was extensive, with special focus on bone marrow status. Using strict criteria, 24 patients (68%) achieved a good partial response (GPR), which comprised normalization of bone marrow, and ten (28%) achieved a partial response (PR), and one progressed. The overall response rate was 96%. Thirty-two patients underwent surgery, and complete macroscopic removal of the primary was achieved in 21 (65%). After completion of induction and surgery, six patients (17%) were in complete remission (CRm), without evidence of any residual disease; nine (26%) were in very good partial remission (VGPRm; same as CRm except persistence of nonpathologically evaluable improved bone scan), and 19 (51%) were in partial remission (PRm). Toxicity was acceptable, and no treatment-related deaths occurred. These results show no substantial improvement compared with those previously reported with similar but nonalternating regimens. We advocate a two-category concept (response, remission) to describe initial therapy results in metastatic neuroblastoma and emphasize the need to assess bone marrow by an extensive evaluation.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1987

[Major beta-thalassemia and bone marrow allograft. Two cases and a study of the literature].

A 5 year 9 month-old boy has received a bone marrow allograft for beta-thalassaemia major. Conditioning included busulfan: 16 mg/kg, cyclophosphamide 200 mg/kg and a (6 Gy) thoracoabdominal irradiation. After a 16 months follow-up, the child is currently in complete remission without treatment with all the markers of his donor. His 9 year-old sister has been allografted for beta-thalassaemia major, with the same conditioning regimen. After engraftment, rejection occurred at day 85 with severe aplastic anaemia. A second graft was performed with the same donor without engraftment and the patient died at day 18 of pneumonitis. A review of the literature is proposed and the ethical choices are discussed.

Bone Marrow Transplantation↗
PubMed1987

[Detection of residual cells contaminating the bone marrow in neuroblastoma. Apropos of 80 bone marrow evaluations].

Eighty bone marrow studies (each including 4 aspirates and 4 trephine biopsies) were performed in 37 children with stage IV neuroblastoma to assess the most accurate means for detection of invasion by neuroblastoma cells. Among 38 abnormal results, only trephine biopsy(ies) were found positive in 24 cases (63%), only aspirate(s) in 5 cases (13%), and both in 9 cases (24%). In 37% of abnormal results, only 1 of the 8 tests performed was found positive. No benefit was obtained from either associated touch imprints of iliac biopsies (67 investigations), or exploration of extra-iliac sites (66 sternums, 53 tibias). Two monoclonal antibodies, claimed to be specific for detection of neuroblastoma cells in bone marrow, were used in 56 investigations; they could detect minimal residual disease in some cases, but they were unreliable when no staining was obtained if initial phenotype of neuroblastoma had not been assessed, or when few isolated cells were observed. Prospective studies using immunocytology and immunohistology are thus warranted.

Antibodies, Monoclonal↗
PubMed1987

[Methotrexate poisoning: value of exchange transfusion].

The authors report on a case of methotrexate (MTX) intoxication occurring in a young boy treated for osteosarcoma. Exchange transfusion was shown to be an effective method. Respective indications of MTX removal methods are discussed: haemodialysis and charcoal haemoperfusion should be proposed for massive and acute intoxications; exchange-transfusion may be proposed as a treatment for prolonged and moderate intoxications.

Acute Kidney Injury↗
PubMed1987

[Fulminating hepatitis A in children. Apropos of 4 cases].

We report four cases of fulminant hepatitis in children (4 to 15 years) who developed an hepatic encephalopathy grade III to IV, 4 to 13 days after the onset of their illness. Three patients recovered without sequelae. The complications were neurological: one child showed elevation of the intracranial pressure, successfully treated after monitoring of extra-dural pressure; one suffered from cerebral death. Hepatitis A was diagnosed by the presence in serum of the IgM component of hepatitis A antibody, but another etiologic factor was present in two cases: an halothane anesthesia and an Epstein Barr virus infection which could explain the severity of the hepatitis.

Adolescent↗
PubMed1986

Persistence of t(4;11) in cytologically normal bone marrow treated ex vivo with Asta-Z.

The chromosomal anomaly t(4;11) is closely related to a specific type of acute leukemia: occurrence in young children, hyperleukocytosis with a particular immunologic phenotype, and poor response to therapy. Allogeneic bone marrow (BM) transplantation has been done in a few cases. We report a case in which a complete remission was obtained after intensive therapy. Because no donor was available, an autologous BM transplantation was performed after purge ex vivo of the BM collection by Asta-Z. Relapse occurred at day 45.

Bone Marrow↗
PubMed1986

Massive therapy and autologous bone marrow transplantation in pediatric and young adults Burkitt's lymphoma (30 courses on 28 patients: a 5-year experience).

A 5-yr experience of massive therapy and autologous bone marrow transplantation (ABMT) for Burkitt's lymphoma is reviewed. Thirty courses were given to 28 patients. Three patients were in resistant relapse and all three died before day 54 post ABMT. Thirteen patients were in non-resistant relapse and seven are alive with non-evidence of disease (NED). All three patients grafted in partial remission (PR) are alive NED including two with initial central nervous system (CNS) disease. Nine patients were grafted in 1st complete remission (CR) either because of long delay to achieve CR or as consolidation in those with initial CNS involvement or leukaemia. Three of these nine are alive including 2/3 with a long delay to CR and 1/5 initial CNS. The overall survival NED for the 28 patients is 46%. The median observation time post ABMT, 22 months. Clear indications for ABMT in BL are in our opinion restricted to about 20% of the patients: non-resistant relapses and PR after initial induction therapy. Massive therapy as consolidation of 1st CR after initial CNS involvement and in resistant relapses should still be considered as experimental. In 14 patients whose marrow was purged there is laboratory evidence suggesting that the purging procedures used in this study may have been incomplete. Purging techniques still require perfection at a laboratory level and their rationale should not be judged on the basis of incomplete procedures.

Adolescent↗
PubMed1986

Histological, cytological and immunological analyses are complementary for the detection of neuroblastoma cells in bone marrow.

On 80 occasions 4 iliac biopsy trephines and 4 iliac aspirations were performed in 37 children with neuroblastoma at various stages of the disease. In 38 of these procedures, tumour cells were detected. In 24% of cases, both trephines and aspirates were positive, whereas in 63% neuroblastoma cells were detected only on the trephines and in 13% only on the aspirates. In addition, in 37% of the stagings, only one out of the 8 investigations was abnormal. Only in one of 33 pathological cases, was BM involvement diagnosed on trephine imprint. No involvement was ever observed on tibial and sternal aspirates without iliac involvement. Immunological studies with two monoclonal antibodies HSAN 1-2 and UJ13A were performed on 56 occasions. Cytohistological and immunological studies were concordant in 39. In 3 studies, the antigens recognized by the two monoclonal antibodies were not expressed by the initial tumour and in 3 additional studies immunological results were falsely negative; but in 11 cases monoclonal antibodies identified residual malignancy despite normal cytohistology. From this study, biopsies appear more helpful to detect malignant cells than aspirates. Immunological staining clearly leads to a better definition of tumour cells in aspirates.

Biopsy↗
PubMed1986

[Chromosome polymorphism in allogeneic bone marrow grafts. Methods and results].

Cytogenetic studies using constitutive heterochromatin variations represent one of the best method in documentation of allogeneic bone marrow transplantation (BMT). Sex mismatched transplants are evaluated by gonosomal examination. In sex matched patients, several banding techniques are performed before BMT to detect autosomal polymorphism in donor and recipient. Can be informative: chromosomes 3 and 4 centromere, acrocentric centromere and satellites, chromosomes 1, 9 and 16 secondary heterochromatic constriction. Authors describe their methodology for these studies and results about 81 patients.

Bone Marrow Transplantation↗
PubMed1986

[Seriousness of severe acquired aplastic anemia in children. Apropos of 32 cases].

Thirty-two cases of severe aplastic anemia in children less than 16 years of age, observed between 1967 and 1985 in the Rhône-Alpes area were studied retrospectively. A toxic cause was suspected in 7 cases, an hepatitis in 5 cases. Patients presented with 2 of the 3 following cytopenias during the 3 weeks following diagnosis: hemoglobin level below 100 g/l, reticulocyte count lower than 20 G/l, neutrophil polymorphonuclear leukocyte count below 0.5 G/l, platelet count lower than 20 G/l. Whatever the treatment, prognosis was poor: 6 surviving patients (18.7%). Nineteen patients did not undergo bone marrow transplantation: only 2 are surviving after 4 years, one after low dose corticosteroid therapy, the other after antilymphocyte globulins and androgen therapy; 10 of 17 deaths occurred in the first 4 months. Thirteen bone marrow transplantations from sibling donors, of which 10 HLA A, B DR identical and 3 haploidentical allowed for 8 prolonged takes but only 4 survivors of whom 2 lasted for more than 2 years.

Anemia, Aplastic↗