Superoxide dismutase deficiency in patients with nitrogen mustard therapy-induced intravascular hemolysis.
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Biomedical subjects
Publications and source records attributed to D Dhermy.
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Using immunodiffusssion tests, the authors describe anti-muscle actin and anti-erythrocyte spectrin auto-antibodies in sera of patients with chronic active hepatitis. These results are compared with study of sera containing anti-striated muscle auto-antibodies and the meaning of these anti-spectrin antibodies is discussed.
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Clinical and histopathologic study of central nervous system (CNS) was performed in 46 acute myeloid leukemia (AML) and 16 chronic granulocytic leukemia in the blastic phase (CGL). Involvement of the CNS developed in 28 cases. Eighteen patients out of these 28 had neurological symptoms. The frequency of meningeal leukemia depends on the number of lumbar punctures and on the survival time. Post-mortem examination was performed on 45 patients. Eighteen had evidence of CNS leukemic infiltration (18/45 arachnoid, 10/31 dura, 5/45 brain). Hemorrhages are frequent even without CNS involvement (19/27). CNS leukemic infiltration is common enough in AML and CGL to justify agressive diagnostic, therapeutic, and prophylactic measures.
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Angio-immunoblastic lymphadenopathy (A.I.L.) was classified as a special type of malignant lymphoma. However, the same morphologic features can be observed in non-malignant diseases. We report a case of A.I.L. associated with Prurigo Strophulus: pseudo-tumoral adenopathy and cutaneous involvement disappeared with parasiticide treatment.
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68 consecutive adults were treated for acute myeloblastic leukaemia (A.M.L). Involvement of the central nervous system (CNS) developed in 15 cases. The clinical characteristics of nine of these patients were: symptoms of increased intra-cranial pressure in 5, cranial nerve lesions in 4, peripheral nerve lesions in 5, cord compression in 1, hemispherical symptoms in 7. Lumbar puncture vas performed systematically on seven patients, two of them with evidence of meningeal leukaemia (M.L.). Post-mortem examination was performed on seven patients who died during the first induction treatment: three had evidence of C.N.S. involvement. M.L. developed in three patients during complete hematologic remission (R.C.) In patients younger than 60 years old, the incidence of M.L. was 8/24 (approximately 33%). Medial survival time was reduced:8 months in cases with M.L., compared to 13.5 months. Cerebro-fluid protein levels were increased in six cases without blast cells: in four cases, M.L. occurred within a few months. The frequency of meningeal leukaemia in A.M.I. is under-estimated. Eradication of this frequent complication is difficult. Prophylactic meningeal treatment may be instituted for patients younger than 60, to reduce the incidence of M.L. and to improve the median remission time.
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Pure rabbit fibrinogen was prepared by a method involving two ammonium sulfate precipitations, one 2 M phosphate buffer precipitation, one DEAE cellulose chromatography and lastly one Sepharose 6 B chromatography. The aminoacid composition was determined and an immunonephelemetric assay was proposed. This assay followed an accurate determination of fibrinogen concentration in a rabbit with inflammatory reaction.
Readily available elements were used to build an automatic apparatus dedicated to the preparation of erythrocyte ghosts. The apparatus is designed around a low-cost re-usable hollow-fiber filtration cartridge (marketed for therapeutic plasmapheresis). The apparatus is controlled by a simple programmer (based on a diode matrix and low cost timers and liquid level sensors): once the apparatus is loaded with whole red blood cells, washing of cells, as well as hemolysis and washing of ghosts, is performed by the machine in about 4.5 h without any operator intervention. Automatic filter cleaning takes a further 110 min.
In the Madagascar Highlands, 0.76% of children from 168 random primary schools, and 19 of 150 families from 3 villages, had oval-shaped erythrocytes. Most harboured the deletion in the band 3 gene characteristic of South-East Asian ovalocytosis. This genetic trait supports the Indonesian origin of the Madagascar settlement.
Hereditary spherocytosis (HS), a common human inherited haemolytic anaemia, is associated with partial deficiency of different erythrocyte membrane proteins. In a subset of dominant HS, a partial membrane expression deficiency of band 3, the erythrocyte anion exchanger (AE1), have previously been characterized, and several mutations in the band 3 gene have been found: amino acid substitutions at conserved positions in the membrane domain, nonsense and frameshift mutations. In HS patients bearing missense mutations, the mutated transcript was present, whereas only the normal transcript was found in HS patients with frameshift mutations. In the former group, the membrane expression deficiency of band 3 was significantly more important than that observed in the latter group of HS patients with frameshift mutations, suggesting that missense mutations may have a dominant negative effect. In the present study, transient and stable transfections of K562 and COS-7 cells were used to demonstrate, by immunoblots of cell lysates and immunofluorescence studies, that the band 3 membrane domain bearing the R490C mutation (band 3Bicetrel) is not targeted to the plasma membrane and is retained in the endoplasmic reticulum. Transient cotransfections of K562 cells with plasmid coding for the normal membrane domain of band 3, together with increasing amounts of plasmid coding for the mutated R490C membrane domain, demonstrated that the band 3 mutant polypeptide exerts a dominant negative effect on the plasma membrane targeting of the normal band 3.
The authors report the clinical and hematological course of 20 patients with polycythemia vera complicated by acute leukemia. This was responsible for 35,8% of the 53 deaths in the series of 103 patients treated with radioactive phosphorus. It occurred on average 12 years after the first treatment. The data in these cases are compared with those in the literature. The role of ionising radiation and/or the myelo-proliferative syndrome itself in the onset of acute leukemia is discussed.
The immune abnormalities observed during acute myeloblastic leukemia often remain undiagnosed for they generally have no practical consequences. The association of peripheral thrombopenia and a positive direct Coomb's test, during two cases of acute myelo-monocytic leukemia should be recognised owing to the difficulty of interpretation and the therapeutic consequences.
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The authors report the case of a patient with lymphosarcoma and erythroblastopenia who died following a surgical operation for gastric carcinoma. The erythroblastopenia, corrected by radiotherapy of the lymphosarcoma, reappeared during the relapse. The link between these two diseases seems undoubted. The later appearance of a gastric carcinoma suggested the predisposing role of the first neoplasm, and/or its treatment, through an immunological disorder.
Three cases of osteosclerotic myeloma are reported. Clues for diagnosis are agarose gel electrophoresis and sclerotic bone biopsy. Review of 50 cases of literature points out frequency of peripheral neuropathy and Ig A type Immunoglobulin. Iliac crest biopsy shows thickening of bone structures and plasmocytosis in medullary spaces. Main X-Ray signs are mentioned and pathogenesis is discussed.