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Biomedical subjects

J Bernard

Publications and source records attributed to J Bernard.

At least 307 records · Page 17Linked to original sources

[Quantitative study of the medullar fibrosis in 84 cases of idiopathic myeloid splenomegaly (author's transl)].

84 bone marrow biopsies were analysed by quantification methods with Classimat. The initial diagnosis was: idiopathic myelofibrosis with splenomegaly. A previous study divided the patients among three categories, according to the classification in three progressive stages of the bone marrow disease. The quantitative lecture was done after reticulin coloration, "in blind", and on several biopsies from the same patient. The results show good correlation with classical histologic methods. The quantitative measure of fibrosis is already important as soon as the first stages of the disease. Correlations with prognosis are more difficult to define.

Biopsy↗

Total body irradiation in bone marrow transplantation. Hôpital Saint-Louis results.

Total body irradiation was used in 22 patients as part of their conditionning regimen for bone marrow transplantation. Nine patients with acute leukemia received 1 000 cGy TBI in addition with chemotherapy. None of them survived and the main cause of death was interstitial pneumonitis (50%). 4 patients received 1 000 cGy with a lung shielding of 500 cGy. Two patients with acute leukemia died of leukemia and sepsis, two patients had aplastic anemia, one is surviving, the other died of severe GVHD and infectious complications. Nine patients with severe aplastic anemia strongly immunized by previous blood transfusions received 800 cGy TBI with a lung shielding of 400 cGy. No rejection was observed and 7 patients (63%) are currently alive. One patient died of interstitial pneumonitis probably related to CMV infection, one of subacute necrotizing hepatitis, two of severe acute GVHD. It is concluded from this study that TBI remains the best immunosuppressive conditioning regimen even in strongly immunized patients. It may be a contributing factor of the incidence and severity of interstitial pneumonitis. A reduction of the dose ot the lung to 400-500 cGy seems to decrease the severity of this complication.

Anemia, Aplastic↗

[Geographical hematology and population dynamics].

Hemotypology, which is based on the study of a large number of immunological and enzyme systems in the blood, has shown the extraordinary polymorphism of the human species and the lack of a genetic barrier between groups once considered as separate races. The typological mode of thought predominated in anthropology until the middle of this century. Mankind was divided into races according to a theoretical profile characteristic of each one, the holotype, which all the members of the same race were thought to resemble. Today we tend toward the substitution of population thinking: the human species, like all the other animal or plant species, is made up of populations, reproductive units whose members are more likely to mate within the group than outside it. A population is never totally closed and it is the interpopulational genetic flux which assures the homogeneity of the species. Three factors play a fundamental role in the genetic structure of human populations: 1. An ancestral genetic heritage from the distant past is modified by external contribution such as genetic flux and hybridization; 2. Chance is an especially important factor in very isolated small groups; 3. Natural selection: the majority of all genetic factors are not neutral, as we used to think, but possess a certain selective value. This nonneutrality doubtless explains the maintenance of the hemotypological polymorphism in man, as in the model proposed by A.E. Mourant and J. Ruffié. Following these ideas, sometimes it is possible to find the hemotypological traces of important events, especially of the great migrations of the beginning of the neolithic or the beginning of the historic period. Examples are cited which concern the peopling of sub-Saharan Africa, the western Mediterranean and western Europe, and of the continental Far East and Japan. This conceptual revolution, based on the dynamic idea of populations and not on that of the typological conception of race, has shed new light on the science of anthropology and has bridged the gap between hematology and history.

Africa, Northern↗

Clinical activity of detorubicin: a new anthracycline derivative.

The anthracycline derivatives are intercalating drugs which are of major importance in the treatment of leukemias and in the management of solid tumors. Structural analogs have been prepared by semisynthetic modifications in an attempt to extend the spectrum of antitumor activity and to reduce toxicity (acute myelosuppression and cardiotoxicity). This report concerns our preliminary clinical experience in 111 patients who received detorubicin. Two dose schedules were used in acute leukemia patients. Sequential doses were active in acute leukemia relapses but the mucous membrane toxicity was excessive; more recently, intermittent doses proved active in acute leukemia relapses (one 6-mg/kg dose) and in a patient with resistant Burkitt's lymphoma. In non-Hodgkin's lymphomas, a complete response rate of 71% was achieved with an intermittent schedule (3 mg/kg/day X 3 weeks). A remarkable shrinkage of skin involvement was also observed. Detorubicin showed a high activity in mycosis fungoides (five regressions among six patients) and some activity in soft tissue sarcomas, osteosarcomas, and various solid tumors.

Acute Disease↗

[Results of the radiotherapy in the non Hodgkin's lymphomas treatment (author's transl)].

In our 98 patients all treated by association chemotherapy + cobaltherapy, 37 cases were stages III and IV and 71 cases were of diffuse pathology classification. Meanwhile the results were not so poor: 66% at 5 years survival (all stages) and 69% for stages I and II. The curves of survival rate generally stabilize at 7 years. The prognostic depend of the pathology class: diffuse LBS and diffuse HBS have an evolution similar to acute leukemia (44.5% and 19% at 7 years survival), on the opposite nodular LBS, diffuse LCS have 100% survival at 7 years. Diffuse cases have visceral relapses (liver, bone marrow, Waldeyer ring) or generalized lymphatic involvement; nodular cases have generally localized and only lymph-nodes relapses. The best results by large fields irradiation suggest a contiguous extension of the disease, but there is also distant lymph nodes relapses. In the nodular cases, relapses can be generally cured by localized radiotherapy. In the diffuse case, we do not know if it is better to give total lymphnode irradiation (or to give more chemotherapy with economic irradiation). 10 gastro-intestinal cases were treated by moving strip technique with excellent tolerance and 67.5% at 5 years survival. These results show that interesting results may be obtained in non Hodgkin lymphomas by association of chemotherapy, with selective and moderately large fields irradiation, even in disseminated cases, even in diffuse cases.

Antineoplastic Agents↗

[A trial of combined chemotherapy and radiotherapy for stage IV Hodgkin's disease with initial lung involvement (author's transl)].

Between 1972 and 1975 a total of 20 patients were treated for stage IV Hodgkin's disease with initial lung involvement. All had pathological findings in the mediastinum, and 17 had a nodular-sclerosing type histologically. Four patients were in full remission after six months of multiple chemotherapy. Further chemotherapy or combined chemotherapy and radiotherapy achieved full remission in a total of eight patients, i.e. a six-year survival rate of 40%. The prognosis was poor if lung involvement was still evident after six-month chemotherapy. But if the lung involvement disappears radiologically the authors recommend further treatment, analogous to that in stages II and III, i.e. exploratory laparotomy with splenectomy, plus upper and - if necessary - lower section radiotherapy.

Adolescent↗

Prognostic factors in chronic granulocytic leukemia. A study of 798 cases.

Between 1959 and 1973 were analyzed the records of 798 patients with chronic myelocytic leukemia. Mean survival (MS) for the entire group is 42 months. 342 patients have been followed closely during and after development of blastic transformation. Presence of following symptoms at the time of diagnosis: asthenia, weight loss, bone pain, fever, sweats and digestive disorders is of poor prognosis significance (MS: 36 months, no sign: MS 75 months) (P less than 0.001). Spleen size is also a prognostic factor. MS are respectively 70, 52 and 35 months if initial splenomegaly is moderate (less than 3 cm), marked (less than 6 cm) or tumoral (greater than or equal to 6 cm). Thrombocytopenia (less than 15,000/mm3 or thrombocythemia (greater than 1 million/mm3) have a poor prognosis with median survival 22 months and 28 months. If peripheral blast cells (hemocytoblasts + myeloblasts) exceed 5%, the prognosis is worse; beyond 10% MS is 26 months. In contrast certain factors have better prognosis: hemoglobin greater than or equal to 14 g/100 ml, young age (less than 20 y.) MS: 62 months), female sex and an initial WBC count below 25 x 10(3)/mm3 (MS: 70 months).

Adult↗

[Survey of anthracyclines derivatives in haematology (author's transl)].

Anthracyclines, such as daunorubicin (DNR), rubidazone (RBD) and adriamycin (ADR) are intercalating drugs used in cancer chemotherapy. They inhibit synthesis of DNA and RNA, break DNA and inhibit mitochondrial oxidative chain. Their antitumoral experimental activities depend upon type of drug, tumor and route of administration. After i.v. administration, the drug is present in all tissues except central nervous system. Its disappearance from the plasma is biphasic with a long terminal half life, justifying intermittent chemotherapy. Anthracyclines metabolism occurs mainly in liver micrososomes, and 90% metabolites are excreted in the bile. The main toxicity is cardiac, as a congestive heart failure which appears when a cumulated drug dose is overcome. In man only, a few derivatives have been studied, compounds with activity and no cardiotoxicity are still in research. Action of malignancies depends on type of derivative. We use DNR since 1967, it is a remarkable active drug in induction treatment of AML, it is the only active drug on acute promyelocytic leukemia, and it increases number of remissions in all of adult patients and severe forms of children ALL. Adriamycin (ADR) is active on solid tumors (osteosarcoma, breast and thyroid cancers) and lymphomas. With rubidazone (RBD) we obtain 2/3 of remissions in acute monoblastic leukemia, and it is easier to use than DNR and equally active on AML. RBD is also active on severe cases of lymphomas (lymphosarcomas and Hodgkin's disease). A new compound DEA 14 DNR seems interesting: experimental antitumor activity is high (compared to DNR, RBD and ADR) and it appears to possess activity on solid tumors in man.

Adult↗

[Hereditary haemorrhagic telanglectasia in a large Saharan familly. 87 cases in the same family (author's transl)].

Study of a large family in the Sahara led to the discovery of 87 cases of hereditary haemorrhagic telangiectasia in six generations. This disease had not previously been described in Arab families. The particular structure of this family and the high incidence of consanguinity made it possible to study one individual who was homozygous for hereditary haemorrhagic telangiectasia gene. Examination of the family tree confirms the autosomal dominant transmission of the disorder.

Algeria↗

[Familial myeloproliferative syndromes. Study of 6 families and review of literature].

Ten clinical observations concerning six families with familial myeloproliferative disorders are reported. Family no. 1 : two brothers, RES with myelosclerosis and ROS with chronic myeloid leukemia. Family no. 2 : PG atypical myeloproliferative syndrome and his brother polycythemia vera. Family no. 3 : DF myelosclerosis and her son (DR) polycythemia vera. Family no. 4 : DM, polycythemia vera, the mother and a sister with splenomegaly. The brother died with myelofibrosis. Family no. 5 : GA and ML, cousins with polycythemia vera. Family no. 6 : MB and ZG, a brother and sister with polycythemia vera. No consanguinity and no toxic, infections or malignant etiology were found in these families. The literature reviewed emphasises the rarity of the familial incidence of myeloproliferative disorders.

Adult↗