Biomedical subjects
Publications and source records attributed to G Schaison.
An apparently different chromosome abnormality was observed in unstimulated blood cultures from an acute non-lymphocytic leukemic child: 11q- with G banding techniques and 17q- with R banding techniques. The abnormality is explained as a t(11;17) translocation, and the discrepancy between the G- and R-band patterns discussed.
A case of false hyperthyrotropinemia was investigated. The serum of the patient contained heterophilic antibodies against rabbit immunoglobulins secondary to immunization with rabbit serum. Some vaccines against viral or bacterial diseases contain animal serum capable of inducing heterophilic antibodies in man. This technical problem can be prevented by the routine addition of control rabbit serum or immunoglobulins to the TSH RIA.
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Blast cells from 24 patients with acute lymphoblastic leukemia of Burkitt's cell type were studied for lymphocyte surface markers. The leukemia cells were of B-cell origin in 23 cases and showed a non-B, non-T phenotype in 1 case. Surface immunoglobulins on blast cells were monoclonal, with a striking predominance of cases with light chains of lambda type. They consisted most often of high-density IgM usually without associated IgD. However, 3 patients had cells with surface IgG and 1 had surface IgA. The blast cells lacked detectable IgG Fc receptors in more than half the patients. Serum immunoglobulins were studied in 15 cases: A monoclonal IgM was found in 5 patients (whose blast cells had surface IgM) and a Bence Jones protein was found in 2 others, both of whom had blasts with surface IgG lambda.
Between 1965 and 1976, 83 previously untreated children aged 15 years and under, with biopsy-confirmed Hodgkin's disease were assessed, treated, and followed-up at Hoôpital Saint-Louis, Paris. Clinical stages were IA-IIA for 59, IB-IIB for 19, and III-IV for 5 patients. Two main types of treatment were used: - monochemotherapy-radiotherapy in 26 patients who received mantle field irradiation followed by monthly vinblastine injections for 3 years; - 57 patients received a combination of MOPP and radiotherapy. The MOPP-radiotherapy treated patients have a significantly better survival and relapse-free survival than the monochemotherapy-radiotherapy treated patients (86.9 p. cent vs 76.1 p. cent and 83.5 p. cent vs 65.4 p. cent). Thirteen relapses were observed after a median complete remission period of 30 months: 6 patients are now free from disease and one is still under treatment. Ten patients died after a 55 months median survival: 7 died from Hodgkin's Disease and 3 from treatment toxicity. No secondary cancer or leukemia has been observed. The main long-term complications of therapy were sterility in male patients, after chemotherapy, and growth defects and disturbances of thyroid functions after radiotherapy.
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Seventy-four patients with acute pure monoblastic leukaemia treated between 1970 and 1978 were studied retrospectively. The disease was usually hyperleucocytic and tumoral in character. It occurred with equal frequency in subjects of both sexes and at all ages, with peaks at the two extremes of life. Rubidazone gave a high percentage (75%) of complete remissions, but the prognosis remained sombre, with a mean survival time of 200 days. The incidence of meningeal relapses was reduced by prophylactic measures at central nervous system level, but gingival and cutaneous relapses were frequent. The possibility of bettering the present modest therapeutic results by more intensive chemotherapy is discussed.
The cases of one hundred and forty-one patients (85 males, 56 females) treated for hyperleucocytic acute lymphocytic leukemia (H-ALL) were reviewed. In all cases the initial white blood cell count was over 100,000/cu mm. One hundred patients (71%) attained complete remission (CR). The median duration of CR was six months and the median survival was nine months for all patients and 11 months for those who attained CR. Age, initial hemoglobin, and the height of initial white blood cell count over 100,000 had no significant prognostic value. Relapses occurred earlier in patients with a mediastinal mass. The results depended on the treatment used. With modern treatment, including more intensive chemotherapy and central nervous system prophylaxis, CR rate increased from 65% to 81% and median duration of CR improved from four months to ten months. The most important prognostic difference was related to the sex: CR rate was higher (78.5% vs. 66%) and median duration of CR and hematological remission was longer for females (nine months vs. six months and ten months vs. 6.5 months, respectively). This difference only appeared with modern treatments, however: before 1972 the median duration of CR was four months for both sexes, and after 1972, it was eight months for males and 17 months for females. This difference could be explained by the site of the first relapse, which was testicular in only 2% of cases before 1972 and 27% (47% of the males who relapsed) after 1972.
In women with previously normal menstrual cycles the hypothalamus-pituitary-ovarian function returns to normal within one month after anovulatory steroids are discontinued, and although the follicular phase is prolonged, the ovulation mechanisms are restored within 3 to 4 weeks. Oral contraception by itself does not produce amenorrhoea, but it may mask possible disorders in the gonadotropic axis. Thus, in many cases post-contraceptive amenorrhoea indicates previous dysfunction of the anterior pituitary, which appears to involve lack of oestradiol positive feed-back and, to a greater extent, excessive negative feed-back. Following oestradiol administration, gonadotropic inhibition is abnormally prolonged beyond the 15th day. Cyclic activity is usually restored by clomiphene citrate. Post-contraceptive amenorrhoea is sometimes accompanied by galactorrhoea. Oestrogens facilitate prolactin secretion by producing hypertrophy and hyperplasia of pituitary prolactin-producing cells, but progestogens probably have an inhibitory effect on these cells, so that prolactin plasma levels are normal or slightly raised during treatment with anovulatory steroids. A pituitary microadenoma should be suspected in the presence of high prolactin levels, and oestrogenic treatments should be stopped. In all cases of amenorrhoea, whether or not they occur after oral contraception, the same etiological investigations should be carried out.
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Nitrogen mustard added at final concentration of 0.008 5 microgram/ml after 1 day to culture of PHA-stimulated lymphocytes increased drastically the level of chromosome breakage in Fanconi's anemia (FA) patients. A clear distinction between FA patients and FA parents or controls was therefore possible. Nitrogen mustard at the same concentration added to the culture since their initiation increased the SCE levels in FA heterozygotes significantly compared with controls, allowing the diagnosis of the heterozygous state.
Cytogenetic studies of three Fanconi's anemia patients are reported, one of the patients having erythroleukemia, the other two preleukemia. Clonal abnormalities were present in all three cases. Partial chromosomal duplication uncommon in other leukemias was observed. Partial duplication of the long arm of chromosome 3 has been observed in the present case of erythroleukemia examined as well as in a previously reported one.
Five patients with Fanconi anaemia have been treated by bone marrow transplantation from HLA identical donors. Only one patient survived for more than 3 years. She is now perfectly healthy with complete haematological reconstitution with chimaerism and disparition of chromosomal abnormalities. In contrast, four patients died of acute severe GVHD soon after grafting. In addition, all had signs of severe cyclophosphamide toxicity. This evolution could be explained by a special sensitivity of FA cells to alkylating agents and may indicate the need to modify the conditioning regimen in FA patients.
The effect of dihydrotestosterone [17 beta-hydroxy-5 alpha-androstone-3-one (DHT) in the negative regulation of LH was studied throughout 3 months of percutaneous administration of this steroid to six normal male volunteers and six patients with primary testicular hypogonadism. In addition, testosterone (T) was administered according to the same protocol to the six hypogonadal patients. Plasma T, DHT, estradiol, sex hormone-binding globulin, and LH levels were measured by RIA before and weekly during treatment. In normal men, plasma DHT rose regularly from 0.5 +/- 0.2 to 3.3 +/- 0.9 ng/ml after 3 months of treatment. Concurrently, T decreased from 5.5 +/- 2.0 to 2.9 +/- 0.3 ng/ml, and sex hormone-binding globulin levels decreased from 1.2 +/- 0.1 to 0.9 +/- 0.05 mg/liter. Mean basal LH levels remained stable between 1.9 +/- 1.3 and 3.1 +/- 1.1 mIU MRC, LH 68/40 per ml throughout treatment. In hypogonadal men after DHT treatment, basal LH levels were not suppressed. In contrast, after T administration, plasma LH decreased from 19.8 +/- 5.4 mIU/ml to normal levels (3.4 +/- 2.7 mIU/ml). From these studies, we conclude that 1) high levels of plasma DHT, maintained over a long period, were unable to lower plasma LH in either normal or hypogonadal patients, and 2) in contrast, during the same length of time, high levels of plasma T were perfectly capable of achieving feedback regulation of LH secretion. From these data, it seems unlikely that circulating DHT plays an important role in the physiological regulation of LH secretion.
The effects of angiotensin have been studied in four adult patients with the simple virilizing form of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. They were treated with hydrocortisone (25 mg/day) throughout this investigation. Plasma ACTH was normal in three cases, and androstenedione was normal in all cases. However, urinary pregnanetriol, plasma 17-hydroxyprogesterone (17 OHP), aldosterone, and renin activity was increased. The patients were then submitted to three protocols: sodium depletion (10 meq Na/day) for 5 days, sodium repletion (200 meq Na/day) for 5 days, and angiotensin infusion (sufficient to maintain a pressor response) for 60 min. Urinary pregnanetriol, plasma 17 OHP, and androstenedione levels increased in all patients after sodium depletion and decreased after sodium repletion. Plasma ACTH levels were not modified by changes in the sodium balance. Furthermore, angiotensin infusion increased aldosterone and 17 OHP plasma concentrations without any change in the plasma ACTH level. This study shows the direct action of angiotensin on adrenal steroidogenesis, at least in 21-hydroxylase deficiency. It confirms that even in the simple virilizing form, combined treatment with glucocorticoids and mineralocorticoids helps to normalize plasma 17 OHP levels.
Seventy-four cases of pure acute monoblastic leukemia (AMol) have been retrospectively studied. All patients were treated at Hospital Saint-Louis between 1970 and 1978. Diagnosis was based on morphological and cytochemical features according to the FAB classification. This type of leukemia occurred at any age and in both sexes, with a high frequency of extramedullary involvements. Hyperleukocytosis was very frequent and was significantly correlated with increased blood and urine levels of lysozyme, with renal failure and hypokalemia, and with coagulation abnormalities. AMol still has a poor prognosis, despite a best remission rate (75%) obtained with rubidazone, since the duration of complete remission was short. Central nervous irradiation prolonged remission and prevented meningeal relapses, while 6 meningeal relapses occurred in the patients not irradiated. The high frequency of the extramedullary relapses, including gum and skin, emphasized the question of persistant blast cell sanctuaries after achievement of bone marrow remissions. A more intensive induction with several drugs active against monoblasts could be more efficient and prolong the duration of complete remissions.