[Legionnaires' disease in Hodgkin's disease].
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Biomedical subjects
Publications and source records attributed to B Dreyfus.
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A female with chronic myeloid leukemia (XX Ph 1 +) in blast crisis (localized to pleura and lymph nodes) was treated by polychemotherapy. After reversion to the chronic phase, and allogeneic bone marrow transplantation, (BMT) was performed. Sixteen months after BMT, no sign of the disease was present (XY Ph 1-).
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Thrombocytopenic thrombotic purpura is a rare condition of controverted pathophysiology. The authors have observed and treated 10 cases between 1974 and 1979. The clinical features were uniform: haemolytic anaemia with schistocytosis (10/10), thrombopenia (10/10), fever (9/10), varying neurological disorders (9/10) which required assisted ventilation in 7 cases. Renal insufficiency was found in 7 patients, but associated with renal lesions only one and never interfered with the course of the disease. All patients received corticosteroids in high dosage and anti-platelet aggregation drugs. That treatment alone cured 3 patients. In addition, 5 patients underwent plasmapheresis, which seemed to be very effective in 2 cases. Five patients died, 3 of whom despite corticosteroids, anti-aggregants and plasmapheresis. Thus, none of these treatments seems to be effective in all cases, and prospective multicentre therapeutic trials are probably required.
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An analysis of prognostic factors was performed on a series of 92 adult patients with acute non-myeloid leukaemia (ANML). Four factors were shown to be correlated with a poor short-term prognosis: (1) Neuropathy involving a single cranial nerve (numbness of the chin) as a presenting feature. (2) A low percentage blast cell infiltration of bone marrow (less than 55%). (3) Cytological L2 type or L3 type of cells according to FAB classification proposals. (4) Increased age. Two factors were shown to be correlated with a poor long-term prognosis: 1) A high level of circulating blast cells; 2) A high tumour burden, as in childhood ANML. These two long-term prognostic factors were interrelated. In addition, our results suggested that myelofibrosis could be a poor long-term prognosis factor as well.
58 patients with early stages of Hodgkin's disease (I, II, III l) were treated by 3 courses of MOPP chemotherapy followed by extended field irradiation. After 31 to 72 months follow up 87,8 p. 100 of patients are alive in complete remission. The study of the group deserves two comments: 1. Adjuvant MOPP polychemotherapy may be useful, only when a complete response is obtained after 3 courses, when it is so a complete remission of long duration may be hoped in nearly 100 p. 100 of cases. 2 After complete remission induced by combined chemotherapy and radiotherapy, maintenance chemotherapy seems to be useless.
The rapid appearance of acute respiratory distress during the course of 25 hyperleukocytic leukemias was associated with the rapid increase of the leukocytosis. The regression of the tachypnea was spectacular when treating hyperleukocytosis by exchange transfusion and chemotherapy. Blood gas studies, although blurred to some extent by in vitro blast consumption of oxygen, showed a hypoxemia with a hypo-or normocapnia. The symptoms seem to be related to the leukostasis by the mechanical obstruction of the pulmonary capillaries. This leukostasis was shown to be responsible for a septal and alveolar oedema. The high frequency of this syndrome during the course of AGL and of acute phase of CGL seems to be linked to the low deformability of the myeloblasts. In CGL at its chronic phase, CLL or even in ALL, the absence of this syndrome could be explained by the greater deformability of the circulating cells. The hyperleukocytic AGL patients which do not have this syndrome are all characterized by a stable or slowly increasing leukocytosis. Thus, this syndrome seems to characterized by hyperleukocytic granulocytic leukemias with a rapid blood leukocyte doubling rate. Treatment in such cases is an emergency.
Twelve cases of Philadelphia chromosome positive chronic granulocytic leukaemia (CGL) in blast transformation have been investigated using ultrastructural peroxidase detection. In all cases, the leukaemic blasts were negative for myeloperoxidase on the basis of standard cytochemistry. In nine cases a variable proportion of blasts contained peroxidase activity detectable only by electron microscopy, permitting definition of their myeloid nature. By their distinct characteristics and localization, different peroxidase activities were recognized. Thus, several types of blasts were identified: megakaryoblasts (MKB), basophil promyelocytes (BPM), myeloid blasts with small granules containing peroxidase (MyB), and proerythroblasts (ProE). MKB were predominant in two cases and present in four cases, mixed with other myeloid blasts. BPM were abundant in one case and present in seven cases. MyB were identified as a majority in four cases. Three cases remained without any peroxidase. It is concluded that ultrastructural detection of peroxidases is of value for the identification of early myeloid blasts. Their high incidence and the simultaneous presence of several myeloid precursors suggest that during the blast crisis the target cell is frequently a pluripotent myeloid stem cell.
An authentic case of Waldenstrom's macroglobulinaemia without a tumoural syndrome is reported, with initial signs of peripheral pancytopenia. Complete and persistent remission appears to have followed multiple sequential chemotherapy. This very satisfactory and, according to data in the published literature, very rare result suggests that this type of treatment should be prescribed for all patients with macroglobulinaemia with poor prognostic clinical and histological characteristics.