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Biomedical subjects

G Bilski-Pasquier

Publications and source records attributed to G Bilski-Pasquier.

At least 19 recordsLinked to original sources

[Value of plasma exchange in the management of acute immunoallergic thrombocytopenic purpura (author's transl)].

Plasma exchanges were performed in 5 patients with acute immunoallergic thrombocytopenic purpura (ITP) because of severe haemorrhages and/or inefficient or badly tolerated corticosteroid therapy. Plasma exchanges were carried out with cell separators and were usually well tolerated. They resulted in very rapid increase in platelet levels with disappearance of auto-antibodies and dramatic reduction of haemorrhages, and they brought about, or accelerated, recovery in 3 patients. One patient was slightly improved. In the 5th patient, who had meningeal haemorrhage, plasma exchange was effective in increasing platelet levels but was unable to prevent a fatal outcome. The main indications for plasma exchange seems to be acute ITP with severe, life-threatening haemorrhages.

Acute Disease↗

[Initial bone marrow involvement in Hodgkin's disease (author's transl)].

Twenty-five patients with Hodgkin's disease and evidence of bone marrow involvement (BM +) were compared with 25 other Hodgkin patients, chosen at random, who had visceral lesions but free bone marrow (BM -). Both groups were observed throughout the same period. The first evaluation showed that bone marrow involvement was more frequent in male patients and that it produced medullary hypoplasia with WBC and platelet counts lower than in BM - patients. However, bone marrow involvement can only be ascertained by medullar biopsy. BM + patients were treated with multiple chemotherapy (mostly MOPP). Positive response was obtained in 64% and complete remission in 50%, as compared with 70% and 50% respectively in BM - patients. There was no significant difference in overall survival rate between the two groups. It remained in plateau at 50.5% after two years in BM + patients and at 63% after 27 months in BM - patients. When complete remission was obtained the survival rate after one year was 90% for BM + patients and 100% for BM - patients. In the absence of complete remission, however, the median survival time was inferior to one year in both groups. The dosage levels and side-effects of chemotherapeutic agents were similar in both groups, but cytopenia was more pronounced in BM + patients.

Adult↗

Lymphoblastic lymphoma/leukemia with convoluted nuclei: the question of its relation to the T-cell lineage studied in 13 patients.

This work is devoted to the analysis of the nature of lymphoblastic lymphoma/leukemia with convoluted nuclei which were initially described by Barcos and Lukes. Ultrastructural, cytochemical, and immunologic features of tumor cells were investigated in patients chosen according to known morphologic criteria. Through results of the E rosette test, the patients were divided into two groups (E+ and E-). In the E+ group, the predominant features were sex (only men), the mediastinal localization, and the focal positivity of the acid phosphatase reaction. Cytotoxicity tests with rabbit antihuman T-lymphocyte anti-serum confirmed the results of the E rosette test in the 3 patients of the E+ group who were tested and were also positive in 2 patients from the E- group (1 of these 2 patients had the characteristics found in the E+ group and can thus be related to this group; the other patient had none of these characteristics). This raises the question of a leukemia arising from a less differentiated T-cell but this interpretation is limited by the specificity of the anti-T-serum. Ultrastructural study defines more precisely the convoluted aspect but does not at present allow a distinction between the two groups.

Adolescent↗

Angiofollicular and plasmacytic polyadenopathy: a pseudotumourous syndrome with dysimmunity.

Two cases are reported of an apparently distinct type of immune disorder. Beginning with mild anaemia and widespread massive lymphadenopathy, the disease progressed to a fatal autoimmune type haemolytic anaemia. Serum investigation showed polyclonal hypergamma-globulinaemia and some autoantibodies. Repeat lymph node biopsies in each case showed hyperplasia within B lymphocyte territory (follicular hyperplasia and polyclonal plasmacytosis with IgG predominance) and atrophy of T dependent areas. Dilatation of lymph sinuses, vascular proliferation, and sclerosis were striking features. This appears to be a new entity, and reasons are given for separating this disease from other pseudotumourous lymph node disorders associated with dysimmunity.

Aged↗

Atypical T-cell leukemia terminating Hodgkin's disease.

A case of Hodgkin's disease is described which developed into a terminal illness characterized by a malignant proliferation of T-cells. The leukemic cells, after optical and ultrastructural analysis, were distinct from those of myelomonocytic, acute lymphoblastic, chronic lymphocytic as well as prolymphocytic leukemia. Their relationship with the T-cell lineage seemed to be confirmed by a highly positive E-rosette test and by cytochemistry which showed focal positivity of acid phosphatase. The importance of this T-cell malignant proliferation is discussed, especially with regard to cellular interactions in Hodgkin's disease.

Acid Phosphatase↗

[Diagnostic and prognostic value of the assay of lysozyme in acute leukemia (author's transl)].

The diagnostic and prognostic value of the assay of lysozyme in serum and urine was appreciated in 184 cases of acute leukemia. The levels were decreased in the lymphoblastic, mainly of the non B-non T type, and undifferenciated varieties, markedly raised in the monoblastic and myelo-monocytic varieties, while in the myeloblastic ones they were found normal, decreased or slightly increased, and, on the average, significantly higher in the well differenciated than in the poorly differenciated types. For a given cytological type, the level of lysozyme is not correlated with the frequency of the induction of complete remission. However, in the acute myeloblastic leukemia, a significantly higher frequency of infection during or after the induction treatment was observed in the cases presenting initially without a raised serum lysozyme level.

Humans↗

[Anaemia induced by vitamin B12 or folate deficiency: diagnostic value of the "dU suppression" test (author's transl)].

The "dU suppression" test, studying thymine-DNA synthesis from deoxyuridine is abnormal in folate and vitamin B12 deficiency, these two vitamins being involved in this reaction. We have done it in 7 cases of anaemia by folate deficiency, 12 cases of anaemia by vitamin B12 deficiency, in 3 cases of combined deficiency, in 3 cases of acute myeloïd leukaemia, 2 cases of sideroblastic anaemia and 2 cases of intoxication by antifolate. This test is very sensitive: it is abnormal even when there is an anaemia related to a vitamin deficiency with only very light morphologic abnormalities and can discriminate the cases, mainly in myelodysplastic syndromes, where folate deficiency is only an epiphenomen in the genesis of anaemia. The abnormalities of the "dU suppression" are quite corrected by added folate derivatives in folate deficiency. However, cobalamin derivatives did not correct completely the test in vitamin B12 deficiency except when a folate compound is also added. The "dU suppression" test becomes normal about one week after starting vitaminotherapy in vitamin B12 deficiency.

Anemia↗

[Hodgkin's disease: the value of prognostic criteria and of the combination of radiotherapy and chemotherapy in localised lymphoid stages (I and II). 94 patients undergoing laparotomy (author's transl)].

Criteria reputed to be of grave prognostic significance were studied in 94 patients suffering from Hodgkin's disease at stages I and II, after laparotomy and splenectomy. The parameters studied (age, general signs, histological type, mediastinal involvement) are less prognostic factors than indications of unrecognised extension of the disease: almost 1/3 of unrecognised lesions in the presence of one of the criteria; almost 2/3 with two or more. Exploratory laparotomy revealed lesions unsuspected on the basis of clinical evaluation only in 10% of patients with no criteria of poor prognosis. The long term prognosis of the disease depends, in fact, not upon the existence of these criteria but upon the method used in initial treatment. Five year survival and cure rates are significantly better in patients treated with combined polychemotherapy (MOPP) and radiotherapy than in those treated with radiotherapy alone. These results would indicate that routine laparotomy should be abandoned in patients with a localised clinical staging if it is decided to begin treatment with a combination of radiotherapy and chemotherapy. So-called prognostic factors could then be used simply to vary the intensity of the treatment prescribed.

Adult↗

T-lymphocyte colonies in the lymphoproliferative disorders.

Human lymphocytes from peripheral blood, bone marrow spleen and lymph nodes were cultured. Continuous phytoheamagglutinin (PHA) stimulation was used, first during a 24 h liquid preincubation, then during a 5 day culture in methylcellulose. In normal donors a rapid colony formation took place, with a mean of 124+/-82 colonies per 1 times 10(5) preincubated lymphocytes. Cells from such colonies were studied by cytology, scanning electron microscopy and rosette formation techniques; arguments favour the hypothesis that these could be T lymphocytes. Neither granulocytes nor macrophages could be grown, and no lymphoid colony formation occurred without PHA stimulation. The same technique was applied to patients with various lymphoproliferative disorders. Significant colony suppression was observed in nearly every case of chronic lymphatic leukaemia; the number of colonies was reduced in some patients with acute lymphatic leukaemia, lymphosarcoma, dysglobulinaemia and Hodgkin's disease. This lymphoid culture method should be applied to a larger number of patients to determine whether it has a classification value and/or prognostic significance. When colonies were grown in pathological states, rosette formation was identical to that of normal donors; colony formation could be due to persisting normal lymphocytes.

Adolescent↗

'Gd(-) Hôtel Dieu': a new G-6PD variant with chronic hemolysis in a Negro patient from Senegal.

A G-6PD deficiency was detected in a Negro patient from Senegal suffering from congenital nonspherocytic hemolytic anemia. The main characteristics of this variant were: profound defect of G-6PD activity in the red cells, decreased immunologic specific activity, fast electrophoretic mobility, decreased Km-G-6P and normal Km-NADP+, normal inhibition by ATP and NADPH, slightly increased utilization of the substrate analogues, slightly biphasic pH curve, high heat lability, subnormal activation energy. The characteristics of this variant being unique, it was called 'G-6PD Hôtel Dieu.'

Adult↗

[The detection of soluble fibrin complexes by a haemagglutination test. Clinical applications (author's transl)].

Testing for soluble fibrin complexes was performed using a sensitive and reliable haemagglutination assay, with red cells sensitized by fibrin monomers. The principle is based on the fact that the monomers linked to red cells and induce their agglutination. This test, used in clinical trials, has revealed the presence of soluble complexes in every confirmed case of acute DIC, but also in Chronic DIC where diagnosis is difficult to establish (negative ethanol gelation test, normal or sub-normal levels of fibrin breakdown products). In Cirrhosis of the liver, the test gives positive results in a non negligible number of cases. Several hypotheses are made to explain why in certain confirmed cases of DIC, low fibrin breakdown products levels are found.

Aged↗

[Increased hematological toxicity of antineoplastic drugs with simultaneous androgenotherapy (author's transl)].

Thirty two patients with malignant lymphoma - mainly Hodgkin's disease - were randomized for simultaneous treatment by high doses of metenolone during MOPP chemotherapy, to reduce its hematological toxicity. The results have shown surprisingly an increased hemato-toxicity in patients receiving androgens, with significantly more marked anemia and thrombocytopenia, reducing the total doses of anti-cancer drugs. This side effect could be explained by a cycling of the hematopoietic stem-cells and call to some caution when androgens are used during cancer chemotherapy.

Androgens↗

[Bone-marrow biopsy in chronic myeloid leukemia. Value of the initial examination].

In 31 patients with chronic myeloid leukemia, the initial bone marrow biopsy constantly showed considerable cell richness. The maturation of the granulocytes was harmonious in 52% of cases, with a predominance of myelocytes in 32% of cases, and groups of myeloblasts in 13% of cases. Eosinophilia was frequent and fairly intense. Fairly numerous loaded cells and Charcot-Leyden crystals were signs of granulocyte failure in the bone marrow. The erythroblasts were reduced, the megakaryocytes increased. Myelofibrosis was present in 45% of cases. This gave a very characteristic histological appearance. It was not possible to draw up correlations between the type of granulocyte maturation and/or the presence of myelofibrosis and, on the other hand, the clinical course of the disease, but the small number of case studied both in our series and in the literature do not permit one to carry out a proper statistical study.

Biopsy↗

[Bone-marrow biopsy in chronic myeloid leukemia. Significance during development].

In 29 patients bone marrow biopsy carried out during the course of chronic myeloid luekemia, permitted the authors to divide up the patients into 5 histologically different groups according to the association of 3 parameters: the richness in granulocytes, the state of the matrix, the degree of leukoblastosis. There was, in most cases, correspondence between the histological appearance and the clinical and laboratory symptoms. This was particularly clear when the patient entered the terminal phase of the disease. 4 of the 5 histological groups had an unfavourable short-term prognosis, i.e., granulocyte hyperplasia with myelofibrosis, aplasia with normal matrix or with myelofibrosis and massive leukoblast invasion.

Adult↗