Complete remission was achieved in 11 of 22 children with acute myeloid leukaemia using at least two courses of a 24 hour infusion of cytosine arabinoside (Ara-C) 10 mg/kg, followed by push injections of daunorubicin (DNR) 45 mg/m2, and adriamycin (ADR) 45 mg/m2. Consolidation therapy consisted of three courses of Ara-C and 6-thioguanine (Tg) and one course of cyclophosphamide (CPA) and ADR. Central nervous system prophylaxis with intrathecal Ara-C was given in all patients and cranial irradiation of five. Maintenance therapy consisted of 5 day courses of Ara-C and Tg given 4 weekly with immunotherapy (BCG) vaccine and subcutaneous leukaemic cells) between courses. Median length of first complete remission was 99+ weeks, and median survival of all patients was 44 weeks. Median survival of remitters was 195+ weeks and non-remitters, 28 weeks. Two patients developed central nervous system disease, one at presentation and the other 46 weeks from presentation. Five patients have ceased therapy and remain in remission from 32 to 142 weeks after ceasing treatment.
Between January 1975 and December 1977, 264 adult patients with acute non-lymphocytic leukaemia entered the Australian National Leukaemia Trial. Of 251 evaluable patients, three induction regimens achieved similar complete response (CR) rates. CROP (cytosine arabinoside, daunorubicin, vincristine, prednisolone) produced CR in 41% of patients, 7 and 3 (cytosine arabinoside, daunorubicin) in 42% and 7 and 3 plus hydroxyurea in 52%. Remission duration and survival were similar when induction regimens were compared. Forty-five patients reaching maintenance therapy were randomised to either chemo-immunotherapy (BCG plus intradermal leukaemic blast cells) or chemotherapy alone. The duration of CR in these two groups was almost identical, though patients receiving chemotherapy alone had prolonged survival (median 161 weeks) when compared to the chemo-immunotherapy group (84 weeks, p = 0 . 07). Institutions with less developed supportive facilities reported lower CR rates (p = 0 . 04). Leucocytosis (greater than 100 X 10(9)/1) and older age (greater than 50 years) were associated with shortened survival. The Trial has failed to show any advantage for this form of immunotherapy.
Summary A foal with primary severe combined immunodeficiency, diagnosed within the first two weeks of life, was maintained with its dam in semi‐isolation. The foal received continuous prophylactic antibiotic therapy, plasma from a sibling hyperimmunised with equine adenovirus vaccine, and intensive general nursing care. A full sibling female was selected as a bone marrow donor on the basis of red blood cell cross‐matching and mixed lymphocyte reactions. Cyclophosphamide was given before two bone marrow transfusions at 35 and 73 days of age. To prevent graft versus host disease graft versus host disease the foal was maintained on methotrexate therapy. Reconstitution was not achieved nor were there signs of graft versus host disease. The foal died suddenly four days after the second bone marrow transfer when 77 days old. It had remained clinically free of any life threatening infectious disease and at necropsy a remarkable degree of freedom from infectious disease was confirmed. The most notable necropsy findings were bilateral nephrosis and myocardial degeneration and fibrosis. The likely cause of death was an electrolyte imbalance, particularly hypokalaemia, which secondarily affected the myocardium. Renal toxicity caused by the cytotoxic drugs, especially cyclophosphamide, may have contributed to the electrolyte imbalance. Résumé Un foal atteint d'une sévère immunodéficience primaire combinée, diagnostiquée au cours des deux premières semaines de la vie, fut confiné avec sa mère dans un demi isolement. Le foal reçut une antibiothérapie prophylactique continue, du plasma prélevé sur un parent d'un type sanguin voisin hyperimmuniséà l'aide d'un adenovirus vaccinal équin et bénificia de soins attentifs. Une femelle très proche sur le plan immunitaire fut utilisée comme donneur de moelle osseuse, après avoir été identifié par des réactions croisées erythrocytaires et par des réactions lymphocytaires. Une administration de cyclophonamide précéda deux transfusions de moelle osseuse à 35 et à 73 jours. Pour prévenir une réaction antagoniste greffon‐hôte le foal fut maintenu sous traitement au méthotrexate. La reconstitution n'eut pas lieu. On ne constata point de signes cliniques de maladie immunitaire. Le foal mourut soudain quatre jours après le second transfert de moelle osseuse, âgé de 77 jours. Il était resté cliniquement indemne de maladie infectieuse grave. L'autopsie confirma cet état par l'absence de lésions imputables à une maladie infectieuse. Les constatations nécropsiques les plus remarquables furent une néphrose bilatérale, une dégénérescence myocardique. La cause probable de la mort fut un déséquilibre électrolytique, notamment une hypokaliémie qui affecta secondairement le myocarde. Une toxicité rénale engendrée par les médicaments cytotoxiques, en particulier le cyclo phosphamide peut avoir favorisé le déséquilibre électrolytique. Zusammenfassung Ein Fohlen mit schwerer primärer, kombinierter Immundefizienz (PSCID) — diagnostiziert innert der ersten zwei Lebenswochen — wurde mit seiner Mutter in Halbis...
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