[Dosage and efficacy of antilymphocyte serum in severe bone marrow aplasia].
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Biomedical subjects
Publications and source records attributed to F Bauters.
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A coagulation inhibitor of the anti-factor VIII: C type was detected in three non-haemophilic male patients aged 75, 70 and 52 respectively. In all three patients antibody titres were low (less than 12.5 Bethesda units initially, less than 20 units subsequently), and a low but detectable level of factor VIII: C persisted (7 to 12 p. 100 in two patients who had severe haemorrhages and 2.100 in the third one). The 3 inhibitors inactivated factor VIII: C with a complex, type II kinetics (Biggs et al.). Strong doses of anti-haemophilic A fractions were biologically effective in one patient but could not stop severe bleeding. Activated plasma fractions were used successfully on several occasions. Once, moderate and repeated doses of anti-haemophilic A fractions resulted in satisfactory correction of factor VIII: C level, and a minor surgical operation could be performed. An immunosuppressive treatment was administered for 3 weeks to one patient and for 3 months to the other two patients. In all three cases the inhibitor disappeared after 5 to 8 months. In non-haemophilic patients with factor VII: C inhibitor the treatment of haemorrhagic episodes must take into account the severity of bleeding, then the usually complex kinetics of the inhibitor; thus it cannot be a direct copy of the treatment used in haemophiliacs with type I inhibitors.
A male patient, aged 23 years, with no notable previous history, was admitted to hospital because of fever, splenomegaly, and pancytopenia. The bone marrow was infiltrated by mature looking histiocytes displaying erythrophagocytosis. Other findings included polyclonal hypergammaglobulinemia, elevated transaminases, hypofibrinogenemia, and hypertriglyceridemia. A diagnosis of virus-associated hemophagocytic syndrome was made and virology studies attributed it to herpes simplex virus type 1. Therapy with acyclovir, high dose i.v. immunoglobulins, and vinblastine was unsuccessful. With the addition of prednisone, a remission, with disappearance of histiocytic marrow involvement, was obtained but was of short duration, and the outcome was fatal.
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The authors report the case of a woman with Löfgren's syndrome who developed renal failure associated with abnormalities of acidification and concentration of the urine. In the absence of a phosphate-calcium metabolic disturbance, these signs were attributed to a granulomatous interstitial nephropathy demonstrated on renal biopsy. These abnormalities regressed completely with steroid therapy. Secondary renal failure due to an isolated granulomatous interstitial nephropathy is exceptionally rare in sarcoidosis and has never been previously reported in Löfgren's syndrome.
A total of 17 patients, 6 female and 11 male (age range 13 to 56 years), received high dose Ara-C for treatment of refractory acute myelogenous leukemia. Ara-C was given at 3 g/m2 twice daily for 6 days as a 1 infusion. 1 patient (with induced acute leukemia) was treated directly, two after failure of a chemotherapy schedule containing the usual dose Ara-C, 12 for first relapse and 2 for subsequent relapse. Maximum follow up is 16 months. Beside hematological toxicity, systemic tolerance was good with no neurological of cutaneous effects. Despite preventive corticoid eyewash, ocular complications occurred in 6 cases, mild and resolvable in 5 of them. The immediate results were as follows: 3 deaths during induction (18%); 6 failures (35%); 8 complete remissions (CR) (47%). After primary chemo-resistance (two cases) failure was always noted. In 3 cases, after less than 12 infusions had been given, 2 failures and 1 very short CR were noted. In 2 patients, when doxorubicin was added to Ara-C, we observed 1 death during induction and 1 failure. Of the patients achieving CR 8 were treated by periodic courses with high dose Ara-C and 4 of them relapsed. The longest failure free duration was 11 months. Median survival duration of the 17 patients is 5 months.
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The 61 observations of primary thrombocythemia described in this report represent approximately 15% of the cases of polycythemia vera recorded by the authors over the same 18-year period. The group includes 35 females and 26 males, with a mean age of 62. The disease is usually discovered on routine blood tests (half of cases), and more rarely because of hemorrhagic or thrombotic manifestations. Splenomegaly is found in one-third of cases. Platelet count is permanently above 800 X 10(9)/l (mean : 1 500 X 10(9)/l); mild hyperleukocytosis (mean : 16 X 10(9)/l) with predominant neutrophil polynuclears is usual but myelemia is not constant (28% of cases) and always very moderate; red cell parameters are normal in three-fourths of cases, while the remaining patients have anemia, either due to iron depletion or not. Reticulinic myelofibrosis, usually minimal, is found in 40% of cases. Medullary karyotype is always normal, without chromosome Ph1. Platelet functional abnormalities are not constant and do not correlate with the magnitude of thrombocythemia. 51 patients (84%) received myelosuppressive therapy, mainly by busulfan or radioactive phosphorus. Most deaths were due to intercurrent causes and only one patient developed acute leukemia. 71% of patients are alive at five years and subsequent decrease in the actuarial survival curve is very gradual.
Acute lymphoblastic leukemia (cytologically typical) was diagnosed in an 18-year-old man presenting with major eosinophilia. Prednisone therapy was initiated but sudden congestive heart failure occurred (left ventricular insufficiency with pulmonary edema). Concomitantly, blood counts became normal. Remission of the leukemic process was obtained while severe mitral regurgitation developed, due to bacterial endocarditis. Successful mitral valvuloplasty was achieved. Remission is still persisting one year after diagnosis.
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We report 4 cases of patients, 5, 16, 31 and 55 years old respectively, with Burkitt type ALL. Three of them presented with a hypoaesthesia of the chin tip. The cytological features of abnormal cells were those of L3 subgroup in the FAB classification; they expressed surface immunoglobulin markers. EBV serology was positive in two cases but the titres did not indicate a recent infection. The translocation t(8;14) (q24;q32) was found in all 4 patients. In one case, it was possible to characterize a clone with a 7 neochromosome (7p+) and a clone with a supernumerary 7 isochromosome i(7q)+; in other respects these two clones showed the translocation t(8;14) and only the latter remained in the terminal phase. In these 4 patients the disease progressed dramatically even when haematological remission was obtained (two cases), predominantly due to early neuro-meningeal involvement.
Transfusional therapy regimens have been investigated in a series of 385 patients treated for acute leukemia (AL). During remission induction, fatal hemorrhages occurred in 0.7% of the patients whereas, during the same time, deaths due to infections were four times more frequent. Platelet concentrates (PC) were usually given on the basis of clinical indications. When the risk of hemorrhage was especially high, as during the induction phase of acute promyelocytic leukemia, prophylactic platelet transfusions seemed to be necessary. Alloimmunization developed in 17% to 52% of the patients depending on their cytologic type and in these cases, PC prepared from single donors had to be used. Among the 57 patients with aplastic anemia (AA), bone marrow transplantation was indicated in 17, and 5 of the 10 grafted patients died from hemorrhages. In AA, as the risk of sensitization to platelet alloantigens was higher, PC prepared from single donors were systematically preferred to standard PC. If bone marrow transplantation was planned, PC from histocompatible donors were ordered.
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Three cases of chronic myelogenous leukemia (CML) were studied, occurring in 22, 43, and 30-year-old-men. Two observations (nos. 1 and 3) concerned typical CML, treated by discontinuous busulfan; in the last patient (no. 2), also presenting in addition with a constitutional deficiency from Hageman factor, diagnosis (Ph 1 chromosome) was based on a moderate leukocytosis with myelemia, spontaneously regressive for more than one year. Chronic phase duration was 17, 16 and 8 months respectively. During the first blast crisis, abnormal cells were rather of granular type in one case (no 1), undifferentiated in the other two. In the three observations, complete remission was easily obtained with prednisolone - vincristine but revealed very brief; 2, 2 and 4 months. Among the three patients a second blast crisis was preceded, in two cases (nos. 1 et 3), by a new CML phase during 3 and 1 months respectively. Treatment was then purely palliative by 6-mercaptopurine and hydroxyurea.
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