[Association of major abnormalities of the upper extremities (phocomelia) and congenital cardiopathy. Extension of the Holm-Oram syndrome].
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Biomedical subjects
Publications and source records attributed to J Bernard.
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224 patients with stage III and IV Hodgkin's disease (H.D.) have been treated by 6 monthly MOPP courses. 190 patients achieved remission and among them there were 109 complete remissions (C.R.) . All patients received vinblastin maintenance combined with "reinduction" courses of MOPP (68 patients) or irradiation (57 patients). At ten years, remission curves are "on plateau" at 72% for those patients who achieved C.R. at 48% for those who had partial remission, and for all patients the ten years survival rate is 53%. The parameters which influence C.R. achievement are age, fever, histology, but the best predictive parameter seems to be lymphogram: diffuse involvement and aspects of cystic storage pattern as seen in "non Hodgkin lymphoma" heralding an unfavorable prognosis. The lymphogram picture should thus be included as parameter of initial classification and treatment active in "non Hodgkin lymphoma" such as Vincristine - Cytoxan - Prednisone - Adriamicin should be tried in these high risk patients.
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A case of acute Mast-cell leukemia was studied. A 39 years old female presenting with a brief history of abdominal pain and attacks of flushing; peripheral blood and bone marrow contained up to 60% of poorly differentiated blasts with clumping of deep purpule granules. Peroxydase reaction stains were negative, chloroacetate esterase were strongly positive. Toluidine blue revealed metachromatic stain. Histamine content of the cells was highly greater than normal but nos heparinoid activity could be demonstrated. These abnormal mast-cells have been investigated with the electron microscope; only the dense particular type of granule substructure was found, without any lamellae component. The cells were temptatively classified as "immature" mast-cell. The disease was interpretated as an acute leukemic variety of systemic mastocytosis.
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