[Inserm ad hoc committee: Recommendations for the management of women with a genetic risk for developing cancer of the breast and/or the ovary]. / Expertise collective Inserm-FNCLCC: recommandations portant sur la prise en charge des femmes ayant un risque d'origine génétique de développer un cancer du sein et/ou de l'ovaire.

BACKGROUND: Almost 10% of breast and ovarian cancer are inherited, and the majority are linked to BRCA1 and BRCA2 germline mutations. Despite the uncertainty, consensus guidelines were defined to assist practitioners', and patients' decisions about the health care decisions to be made. METHODOLOGY: The ad hoc committee consisted of 14 experts designated by the French National Institute for Health and Medical Research. They all attended eleven workshops at which a systematic analytical review of more than 3,500 articles was carried out. Five additional experts critically analyzed the first version of the report. PROCESS: Two thresholds were defined on a probability scale giving the risk of developing breast or ovarian cancer, to serve as a means of deciding as whether an intervention is worthwhile. The first threshold is that above which an intervention can be envisaged or recommended; the second is that under which an intervention can be ruled out; between the two, the decision has to be made on a each by case basis. SCREENING AND PREVENTIVE STRATEGIES ANALYZED: About breast cancer: 1) hormonal interventions; 2) primary prevention (diet, family planning and chemoprevention); 3) screening (breast self-examination, clinician breast examination, tumor markers, imaging); 4) prophylactic mastectomy. About ovarian cancer: 1) hormonal stimulation; 2) screening (clinical screening, ultrasound and tumor markers); 3) prophylactic oophorectomy. MAIN CONCLUSIONS: With each strategy the following points were dealt with: the information to be delivered to the consult and, the procedure and the indications. The committee's opinion about BRCA mutation screening is that population-based or even large scale implementation are not justified. The committee feels that specific management is indispensable and advocates the use of defined and evaluated procedures, and involvement in clinical trials.

[INSERM-FNCLCC collective expertise. Recommendations for medical management of women with genetic risk of developing breast and/or ovarian cancer]. / Expertise collectice INSERM-FNCLCC. Recommandations portant sur la prise en charge des femmes ayant un risque d'origine génétique de développer un cancer du sein et/ou de l'ovaire.

BACKGROUND: Almost 10% of breast and ovarian cancer are inherited, and the majority are linked to BRCA1 and BRCA2 germline mutations. Despite the uncertainty in the management of women gene carriers, consensus guidelines were defined to assist practitioners', and patients' decisions about the health care decisions to be made. METHODOLOGY: The Ad Hoc Committee consisted of 14 experts designated by the French National Institute for Health and Medical Research. They all attended eleven workshops at which a systematic analytical review of more than 3500 articles was carried out. Five additional experts critically analysed the first version of the report. CRITERIA AND DECISION PROCESS: Two thresholds were defined on a probability scale giving the risk of developing breast or ovarian cancer, to serve as a means of deciding as whether an intervention is worthwhile. The first threshold is that above which an intervention can be envisaged or recommended; the second is that under which an intervention can be ruled out; between the two, the decision has to be made on a each by case basis. STRATEGIES ANALYZED: About breast cancer: 1) hormonal interventions; 2) primary prevention (diet, family planning and chemoprevention); 3) screening (breast self-examination, clinician breast examination, tumor markers, imaging); 4) prophylactic mastectomy. About ovarian cancer: 1) hormonal stimulation; 2) screening (clinical screening, ultrasound and tumor markers); 3) prophylactic oophorectomy. MAIN CONCLUSIONS: With each strategy the following points were dealt with; the information to be delivered to the Consultant, the procedure and the indications. In addition, the Committee's opinion about BRCA1 and BRCA2 mutation screening is that population-based or even large scale implementation are not justified. Although no scientific evidence is available, the Committee feels that specific management is indispensable and advocates the use of defined and evaluated procedures, and involvement in clinical trials.

Recommendations for medical management of hereditary breast and ovarian cancer: the French National Ad Hoc Committee.

BACKGROUND AND PURPOSE: Almost 10% of breast and ovarian cancers are familial, and the majority are linked to BRCA1 and BRCA2 germline mutations. Despite uncertainty about the management of female gene carriers, consensus guidelines have been established to assist practitioners and consultees in making health care decisions. METHODOLOGY: The Ad Hoc Committee was composed of 14 experts appointed by the French National Institute for Health and Medical Research, all of whom attended eleven workshops at which more than 3500 articles were systematically analyzed. Five additional experts critically analysed the first version of the report. CRITERIA AND DECISION PROCESS: On a probability scale of the risk of developing breast or ovarian cancers, two thresholds were defined for use in determining whether an intervention would be worthwhile. The first is the threshold above which an intervention can be envisaged or recommended, and the second is the one below which an intervention can be ruled out; between the two, the decision has to be made on a case-by-case basis. SCREENING AND PREVENTIVE STRATEGIES ANALYZED: With respect to breast cancer: 1) hormonal interventions; 2) primary prevention (diet, family planning and chemoprevention); 3) screening (breast self-examination, clinician breast examination, tumor markers, imaging); 4) prophylactic mastectomy. With respect to ovarian cancer: 1) hormonal stimulation; 2) screening (clinical screening, ultrasound and tumor markers); 3) prophylactic oophorectomy. MAIN CONCLUSIONS: For each strategy the following points were addressed: the information to be given to the consultee, the procedure and the indications. In addition, the committee's opinion about BRCA1 and BRCA2 mutation screening is that population-based, or even large-scale, implementation are not justified. Although no scientific evidence is available, the committee feels that specific management is indispensable and advocates the use of defined and evaluated procedures, and participation in clinical trials.

[INSERM-FNCLCC collective expert's report. Recommendations for management of women having a genetic risk of developing breast and/or ovarian cancer. National Federation of Centers of the Fight Against Cancer]. / Expertise collective INSERM-FNCLCC. Recommandations portant sur la prise en charge des femmes ayant un risque d'origine génétique de développer un cancer du sein et/ou de l'ovaire.

BACKGROUND: Almost 10% of breast and ovarian cancer are inherited, and the majority are linked to BRCA1 and BRCA2 germline mutations. Despite the uncertainty, consensus guidelines were defined to assist practitioners', and patients' decisions about the health care decisions to be made. METHODOLOGY: The Ad Hoc Committee consisted of 14 experts designated by the French National Institute for Health and Medical Research. They all attended eleven workshops at which a systematic analytical review of more than 3500 articles was carried out. Five additional experts critically analyzed the first version of the report. PROCESS: Two thresholds were defined on a probability scale giving the risk of developing breast or ovarian cancer, to serve as a means of deciding as whether an intervention is worthwhile. The first threshold is that above which an intervention can be envisaged or recommended; the second is that under which an intervention can be ruled out; between the two, the decision has to be made on a each by case basis. SCREENING AND PREVENTIVE STRATEGIES ANALYZED: About breast cancer: 1) hormonal interventions; 2) primary prevention (diet, family planning and chemoprevention); 3) screening (breast self-examination, clinician breast examination, tumor markers, imaging); 4) prophylactic mastectomy. About ovarian cancer: 1) hormonal stimulation; 2) screening (clinical screening, ultrasound and tumor markers); 3) prophylactic oophorectomy. MAIN CONCLUSIONS: With each strategy the following points were dealt with: the information to be delivered to the consultant, the procedure and the indications. The Committee's opinion about BRCA mutation screening is that population-based or even large scale implementation are not justified. The Committee feels that specific management is indispensable and advocates the use of defined and evaluated procedures, and involvement in clinical trials.

[Psychological consequences in organ transplantations]. / Conséquences psychologiques des transplantations d'organes.

Organ transplantations constitute therapeutic advances for some fatal diseases. Their specificity are: severe prognosis, waiting for an alive donor (kidney or marrow transplantations) or a dead one, uncertainty of results or side effects. Identity troubles, debt towards the donor characterize also transplantations. They realize a traumatic situation difficult to communicate and demand a long working through psychic procedure. We shall describe these patients' defense mechanisms and those of their family and care takers--the psychologist's role in transplantation units.

The child conceived to give life.

Bone Marrow Transplantation (BMT) demands a living compatible donor. Some parents will conceive a child "for BMT". These pregnancies have three main characteristics. The traumatic situation of the impending death of a child, the inevitable uncertainty of their issue, of BMT results, the intrusion of biology into the family dynamics.

[Host-graft interactions after allogenic bone marrow transplantation; choice of donor]. / Interactions hôte-greffon au cours des greffes de moelle osseuse allogéniques; choix du donneur.

The complexity of host-graft interactions after bone marrow transplantation explains the criteria of choice of the donor. Graft rejection, graft-versus-host disease, graft-versus-leukemia, delayed immune reconstitution, often associated or mutually exclusive are observed even with an HLA identical sibling donor. Syngeneic transplants may be complicated by leukemic relapse related to the loss of the graft-versus-leukemia effect. The compilation of files of unrelated donors increases the possibility of finding a matched unrelated donor. Preliminary results suggest that clinical results may be equivalent to those achieved with HLA identical sibling donors. Overall results of HLA mismatched transplants are disappointing because of the increase of immunological complications. Attempts at preventing graft-versus-host disease with T cell depletion led to an increase of immunological complications: rejection and leukemic relapse. Modifications of the conditioning regimen and use of in vivo monoclonal antibodies are currently being investigated. Ethical, legal, and psychological considerations have also to be taken into account in the donor choice especially with the increase of the donor pool.

Acute lymphoblastic leukemia in very young children. Diagnostic and therapeutic aspects of 43 cases.

Between 1974 and 1982, 43 children less than 2 years of age were treated in the hematology department of Hospital Saint-Louis for acute lymphoblastic leukemia (ALL). Of the patients who presented before 18 months of age, 80% had a WBC greater than 100,000 microliter and/or a great tumor bulk. As a result of our experience, treatment regimens have been changed here from conventional chemotherapy to a very intensive program with a heavy induction (vincristine, daunorubicin, cyclophosphamide, prednisone, and L-asparaginase) and monthly reinductions with the same drugs plus ArA-C, without maintenance. Prophylaxis included CNS irradiation (16-24 Gy) after 12 months of age, plus intrathecal methotrexate. Complete remission (CR) occurred in 78% before 18 months and in 100% between 18 and 24 months of age at diagnosis. In this report the probability of a prolonged CR (33% at 2 years) was the same before and after 12 months of age. However, younger patients were more intensively treated. The prognosis for children less than 1 year of age who received very intensive chemotherapy has greatly improved, with a significantly higher probability of long CR (p less than 0.02). Presently, 10 of 43 children are in CR 27 months to 8 years after diagnosis. Of 18 patients aged less than 1 year at diagnosis, four are in CR. No relapse occurred after 23 months. None of these patients presented with important sequellae, with the exception of one child who suffered from severe bacterial meningitis. An aggressive chemotherapy program is indicated in patients less than 2 years of age. The feasibility of this mode of treatment in young patients is possible only with the help of specific supportive care.

[Children born of leukemic parents. Apropos of 23 children]. / Les enfants nés de parents leucémiques. A propos de 23 enfants.

We have studied the children born of leukemic parents who treatment had stopped. In total, 8 women (3 acute myeloblastic leukemias and 5 acute lymphoblastic leukemias) who gave birth to 11 children, and 6 men (all with acute lymphoblastic leukemias) who fathered 12 children were studied. Of these 23 children, two have a severe congenital malformation, one congenital hypopituitarism associated with mid-line defect, and one laparoschisis, and also two benign abnormalities were observed. The children with abnormalities had a leukemic mother, whilst no leukemic father had an abnormal child. It is well known that the toxic effect of chemotherapy is different in the male and the female gonad. These results are compared to those in the literature, and at present it appears difficult to form a clear opinion on the delayed teratogenic effect of chemotherapy. Fecundity and the risk for future generations are unknown. The opening of an international registry would be useful.

[Complete remission of acute leukemia for more than seven years. A report on 100 children (author's transl)]. / Bilan de 100 enfants en rémission complète d'une leucémie aiguë depuis plus de 7 ans.

A total of 1200 children were treated for leukemia between 1958 and 1971, and 100 of these cases have had an uninterrupted remission for more than seven years: 10 children who relapsed at an early stage have had no further relapses; 93 are alive and in remission after discontinuation of treatment for between 1 and 12 years. Five of the children relapsed and four of these are in remission again. Two children died, one from a hepatocarcinoma and the other after cardiac failure of late onset. A study of these children showed that: 1 - growth rate was normal, 2 - puberty occurred at a normal age, and procreation was possible as 10 children were born from parents who had recovered after having leukemia, 3 - school-work and subsequent social and occupational behaviour were satisfactory, 4 - late psychological repercussions were absent or mild. Long-term prognosis appears to be good, and no other blood disorder has been reported in those cases that have recovered from the leukemia.

[Acute leukemia in childhood: present status of 100 cases after 7 years of complete remission (author's transl)]. / Leucémie aiguë. Bilan de 100 enfants en rémission depuis plus de 7 ans.

Amongst 1,200 leukemie children treated between 1958 and 1971, 60 are in complete remission for more than 10 years and 100 for more than 7 years. There were 96 acute lymphoid and 4 acute myeloid leukemias. Ten patients who have relapsed in the past have not done so lately. The F/M sex ratio is 1.5. Poor prognostic features were initially absent in 2/3 of cases. In 1/3 there was associated hyperleucocytosis and/or tumours. 93 children are in remission, their treatment having been stopped for 1 to 12 years. Five children relapsed and 4 are in a second remission for more than 2 years. Two children died in remission: one from a hepatocarcinoma and one from cardiac failure. These patients have been shown to have the following: 1) normal growth; 2) normal puberty: 8 patients have been able to reproduce, giving 10 children, one with multiple malformations; 3) school achievement and later socioprofessional behaviour has been normal. The patients have often sought a medical or paramedical career. Sequelae are minimal, psychological problems being minimal in the child. With the protocols used, mean remission curve shows a plateau after 9 years and complete definitive care is achieved in 92 per cent of patients surviving at 7 years. The very distant future outlook is not known. No other malignant haematological disease has occurred but one child died from a carcinoma.