HEREDITARY NoN - SPHEROCYTIC HEMOLYTIC ANEMIA WITH IMPAIRED UTILIZATION OF AlT AS WELL AS OF A METABOLIC ANOMALY OF RED CELLS WITHOUT CLINICAL IMPORTANCE
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چکیده
The II Department of Pediatrics, University Medical School, Budapest, Hungary. Or ’. Hetil. 110:713-720, 1969. Structural chromosomal aberrations ( breaks, endomitoses ) characteristic of Fanconi’s anemia were demonstrable in two cases. The red cell AlT level in both cases and hexokinase activity in the first one were normal. Thus, no correlation between chromosomal aberrations and disturbances of red cell metabolism could be found. In vitro study with tetrametansulfonyl-n-mannitol revealed increased fragility of chromosomal structures explained by the changed structure of DNA. The increased chromosome fragility is probably an important symptom in Fanconi’s anemia because these patients have increased susceptibility to leukemia and malignant tumors. No aberration was found in the two fathers (supposed to be heterozygotes ) . The serious pancytopenia could not be influenced by intravenous administration of phytohemagglutinin.-S.R.H. BONE MARROW HISTOLOGY IN VAQUEZ’S DISEASE AND THE PROBLEM OF MYEL0SCLEROSIS. G. Duhamel, A. Najman and R. Andr#{233}. From Service d’Hematologie du Pr. R. Andr#{233},H#{244}pital Saint-Antoine, 53, boulevard Diderot, F.75-Paris, 12e, France. Nouv. Rev. Franc. Hemat. 10: 209-222, 1970. Bone marrow histology was done in 52 cases of polycythemia vera. In 19 patients cellular hyperplasia existed; in these cases the spleen was usually only moderately enlarged and showed no signs of myeloid metaplasia. In 26 patients, the bone marrow showed cellular hyperplasia together with intensification of the reticulin network, but normal architecture. In these cases, notable splenomegaly was present and myeloid metaphasia ‘as very probable. In seven patients, a fibrotic transformation of the bone marrow was observed, besides an increase of reticulin fibres. A true osteomyelosclerotic transformation, as in cases of primary myelosclerosis in the course of polycythemia vera is rare, however, in the experience of the authors.-M.C.V. CAPILLARY MICROSCOPIC INVESTIGATIONS IN PATIENTS WITH POLYCYTHEMIA VERA. P. Dar#{243}czy, Gy. Nagy. The Department of Dermatology, University Medical 813 School, Debrecen, Hungary. Orv. Hetil. 110:593-595, 1969. Capillary microscopic investigations were performed in 35 instances in 25 patients suffering from polycythemia vera. In the periods of exacerbation ( 19 investigations) vasodilatation, relative increase in the number of small blood vessels and a significant decrease of circulation time were observed. These anomalies partially regressed during remission ( 16 investigations).-S.R.H. EVALUATION OF THE THERAPEUTIC EFFECT OF MYELOBROMOL ON POLYCYTHEMIA VERA BASED ON FIVE Yi RS’ OBSERVATION. J. Szentkl#{225}ray. From the Department of Medicine, National Oncopathological Institute, Budapest, Hungary. Orv. Hetil. 110:651-653, 1969. The therapeutic effect of Myelobromol in 23 patients suffering from polycythemia vera during a period of 5 years was investigated by 63 treatments. Hematological and dinical symptoms showed complete remission in each case. In relapse, complete remission was obtained by repeated treatment with Myelobromol. The average duration of remission after the first treatment was 14.7 months, and 6-9 months following repeated treatments. A dose of 250 mg. per day of Myelobromol, totaling an average dosage of 10,000 mg. in the first treatment and 8250 mg. in the following treatments, was necessary for obtaining remission. Hematologic studies revealed no considerable side effects.-S.R.H. OVALOCYTOSIS ASSOCIATED WITH MALIGNANT THYMOMA. R. F. Platzer. Clifton Springs Hospital and Clinic, Clifton Springs, N.Y. N. Y. State J. Med. 4:30-432, 1970. A 44-year-old white male with malignant thymoma developed ovalocytosis. Red cell agenesis and ovalocytes developed after thymectomy and were associated with recurrence of the malignant thymoma. There was no response to cortisone, chiorambucil or testosterone. It is postulated that the ovalocytosis was a manifestation of the disturbed erythropoiesis with malignant thymoma.-J.E.U. EFFECT OF AMBERLITE AND McCO3 ON IRON-CONALBUMIN AND IRON-TRANSFERBIN COMPLEXES. J. Balasch and J. Planas. For personal use only. on October 23, 2017. by guest www.bloodjournal.org From
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تاریخ انتشار 2005