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R Leblay

Publications and source records attributed to R Leblay.

At least 37 records · Page 2Linked to original sources
PubMed1990

[Treatment of autoimmune neonatal thrombocytopenia with high-dose intravenous immunoglobulins].

During the neonatal period, 6 infants born to mothers with auto-immune thrombocytopenia purpura were diagnosed as having severe thrombopenia (platelet counts below 30 X 10(9)/l). High doses of intravenous immunoglobulins (IVIG) (0.4 g/kg/injection for 2 infants and 1 g/kg/injection for 4 infants) were administered as of the first week of life. Short-term efficacy was good in all cases (platelet counts over 50 X 10(9)/l, 2-10 days after the first injection). During the second week of life, 5 newborns had relapses which were rapidly reversed by one or several IVIG injections. The thrombopenia was cured between days 19 (shortest) and 41 (longest) and, more over, no side effects were observed. High doses of IVIG seem to be an effective and well-tolerated treatment for neonatal auto-immune thrombopenia, and they can be recommended over classical treatments (corticotherapy, exchange transfusion, platelet transfusion) as a first line of attack.

Adult↗
PubMed1990

[Diagnostic splenectomy. Apropos of 38 cases].

The authors report a retrospective study of 38 splenectomies performed as a diagnostic procedure. In the first group, the diagnosis was made by surgery: 27 cases (71.1%) included the usual large number of lymphomas. In the second group, there was no conclusion: 11 cases (28.9%), patients were younger and the spleen was lighter with a statistical significance (p less than 0.006 and p less than 0.003). Three of the 11 patients in the second group subsequently developed a malignant blood disease. Morbidity was high: out of 38 cases, 14 complications occurred in 11 patients (28.9%). One patient died on the fifty fifth postoperative day. The authors consider that splenectomy for undiagnosed splenomegaly is an effective procedure for diagnosing lymphoma, but not without risk. Indications for surgery must be carefully evaluated. It is more questionable in young, asymptomatic patients or in the case of moderate splenomegaly. After surgery, with no histologic diagnosis only a long-term survey will differentiate patients with malignant blood diseases from patients with idiopathic splenomegaly.

Adolescent↗
PubMed1989

[Secondary paraneoplastic polycythemia of testicular cancer].

The authors report the case of a 41-year old patient with polycythaemia and a mixed testicular tumour (seminoma with choriocarcinomatous contingent). The demonstration of a raised erythropoietin level in the tumor tissue with a high gradient in relation to the serum argues in favour of secondary paraneoplastic polycythaemia. With a follow-up of 5 years, the patient is still in complete remission from his two diseases. This case may represent the first case of testicular tumour responsible for secondary polycythaemia.

Adult↗
PubMed1988

[Splenectomy in the treatment of idiopathic thrombopenic purpura in adults. Apropos of 43 cases].

Case reports of 43 patients with idiopathic thrombocytopenic purpura (ITP) operated upon from 1975 up to the present were analyzed. After revision of therapeutic indications the predictive prognostic factors for splenectomy, essentially the initial response to corticotherapy, were studied. Findings indicated that splenectomy is effective treatment for ITP since permanent total remissions were obtained in 76% with a morbidity of 16% and a mortality of 2%. Emphasis is placed on the need for minute pre-, intra- and post-operative precautions.

Adrenal Cortex Hormones↗
PubMed1986

Multiple myeloma in two brothers. An immunochemical and immunogenetic familial study.

When multiple myeloma was diagnosed within 6 months in two brothers a family study was carried out in 34 relatives to assess the genetic factors involved. The monoclonal immunoglobulin isotype identified was identical for the two brothers (IgG kappa) as well as their genotype (a = A2B12BfSDR4 GIo2/d:A9B27BfSDR2GIol). Blood protein electrophoresis and the major histocompatibility complex markers (HLA A, B, DR, Bf, glyoxalase phenotypes) were also determined in the other family members. The immunochemical study revealed no other case of monoclonal gammapathy, but 12 cases of low gamma-globulin and three cases of polyclonal hypergammapathy were found. The immunogenetic study showed that no other family member had the a/d genotype of the two brothers, whereas nine family members were semi-identical for haplotype a and five for haplotype d. It is unlikely that a double immunochemical and immunogenetic identity in two siblings with multiple myeloma would be due only to random encounter, rather this finding suggests that, besides environmental factors, genetic factors may be involved in the pathogenesis. Systematic immunochemical and immunogenetic studies in familial multiple myeloma are proposed as a method to further elucidate an eventual genetic background in multiple myeloma.

Agammaglobulinemia↗
PubMed1986

[Multiple bone localizations in hair cell leukemia. Efficacy of treatment with alpha 2 interferon].

We report a case of hairy cell leukemia with multiple bone lesions (skull, tibia, spine, femoral head) clinically, radiologically, and histologically documented. Radiotherapy failed to improve tibial pain. Treatment with alpha interferon resulted in bone pain improvement. Diagnoses and therapy strategies of these unusual features are discussed with respect cases to previously published in the literature.

Bone Diseases↗
PubMed1983

Adherence properties of hairy cells.

The Hairy Cells (HC) from four patients with Hairy Cell Leukemia (HCL) were studied. These cells exhibited membrane properties of B-lymphocytes; quantitation of surface membrane immunoglobulins by the peroxidase labeled antibody technique showed an average number of 20,000 to 40,000 immunoglobulin molecules per marked cell. Observations by scanning electron microscopy (SEM) showed that HC have a great capacity to adhere quickly to glass coverslips, to phagocytose latex particles, and to resist trypsin treatment. These cells possess numerous inter cellular connections forming a network on the glass-coverslip, and thin cytoplasmic projections enabling them to adhere. When cells adhering to a support are stimulated by latex particles, they are able to phagocytose these particles and this results in the swelling of the cell due to the unpleating of numerous folds of the membrane. After this stimulation, HC appeared smooth. Our SEM analysis provides an explanation of the disappearance of the hairy appearance classically described. After trypsin treatment, the adhering HC remain on the glass even if they have suffered membrane damage. In conclusion, SEM examination of adhering cells and phagocytosis demonstrate the variability of the membrane aspect of the HCL cells under various culture conditions.

Aged↗
PubMed1979

[Quantitation of myelofibrosis in blood diseases by electronic image analysis (author's transl)].

Normal and pathologic reticulin networks colored black by silver nitrate can be automatically quantitated by electronic image analysis. By using this technique, different parameters can be obtained, such as the average density, the surface of network meshes, the thickness of the fibers, the complexity of the reticulum, and the heterogeneity of the myelofibrosis distribution. All of these parameters were obtained in 83 osteomedullar biopsies of blood diseases (primary splenomegaly, chronic myeloid leukemia, polycythermia vera, acute leukemia, and aplastic anemia). We have shown that there is no relation between the different parameters obtained and the medullary richness, hematopoietic center, or patient survival. On the other hand, the histomorphometric parameters can be used to distinguish acute leukemia and chronic myeloid leukemia myelofibrosis, while the parameters in primary splenomegaly are shown to be very heterogeneous.

Acute Disease↗
PubMed1977

[Hodgkin's disease: study of delayed hypersensitivity of 64 non treated patients. Value of epicutaneous test using croton oil].

Cellular immunity in 64 patients with Hodgkin's disease was studied during diagnosis before any treatment. The functional deficiency of the thymodependent lymphocyte was demonstrated in vivo by skin tests and, in vitro, by the test of inhibition of leucocyte migration to phytohemagglutininin and spontaneous rosette formation, to sheep red cells. This deficiency correlates with the clinical spread and the histological severity. The relationship between skin anergy and biological tests was found. The epicutaneous test using croton oil possesses non-specific inflammatory activity. It is negative this test is negative, skin reactivity to specific antigens is reduced. The croton tests help interpretation of skin responses to specific antigens and thus assessment of the immune state.

Adolescent↗