Intravascular Hemolysis Associated with Coarctation of Aorta

S 813 vere normochromic normocytic anemia at, or shortly after birth. Karyotyping was normal and the only other laboratory abnormality was an increased fetal hemoglobin concentration. The anemia seemed to remit somewhat with increasing age so that transfusion need was minimal. In addition, one patient may have had a response to steroid therapy.-J. B. S. A NEW HEMOGLOBIN : HB J SABDEGNA ( a50 HISTIDINE *‘ ASPARTIC Acm ) . W. Tangheroni, G. Zorcolo, E. Gab and H. Lehmann. From the Paediatric Clinic, Cagliari University, Italy and the M.R.C. Abnormal Haemoglobin Research Unit, Biochemistry Department, Cambridge University, Cambridge, England. Helv. Paediat. Acta 24:174-182, 1969. A new hemoglobin variant is described. The variant was found in six members of one family; it does not seem to cause anemia nor does it interfere with /3 thalassemia, present concomittantly in two members of the pedigree. The hemoglobin was discovered in a young girl suffering from chronic, nonhemolytic anemia, which could not be associated with the hemoglobin variant.H-I. H. ULTRASTRUCTURAL AND MICROSPECTROPHOTOMETRIC STUDIES OF THE Rim CELL INCLUSION BODIES IN UNSTABLE HAEMOGLOBIN DISEASE. S. R. Holl4n, J. G. Szele’nyi, C. Lelkes, H. Berzy, S. Farag#{243} and Gy. Rappay. From the Central Institute of Hematology and Blood Transfusion and Institute of Experimental Medicine, Hungarian Academy of Sciences, Budapest, Hungary. Haematologia 2:291312, 1968. Case reports, biochemical, microspectrophotometric and ultrastructural findings of two patients with unstable hemoglobin disease are presented. Characteristics of the inclusion bodies brought about by the intrinsic instability of the hemoglobin molecule are compared with those of the inclusion bodies resulting from the extrinsic effect of exposure to phenyihydrazine. In the splenectomized patient very high percentage of Heinz-body containing reticulocytes and erythrocytes could be detected even in the Romanovsky stained blood films. In the blood smears treated with the acid elution technique much less inclusion bodies could be detected in the fetal cells. The big inclusion bodies could not be eluted from the adult red cell ghosts due to the firm adherence of the denatured unstable hemoglobin to red cell membrane. Non-electrolyte permeability was decreased, the methemoglobin level and the rate of methemoglobin formation were elevated in both patients. The unstable hemoglobin fraction could be separated from Hb A by heat precipitation in both patients. The Unstable Hb fraction of Case 2 could also be detected by starch gel electrophoresis as an abnormal fraction just preceding Hb A2. The results of the microspectrophotometric analyses proved that inclusions contain only a hemoglobin-like substance and no nucleotides or nucleic acids. According to our ultramicroscopic results the most conspicuous ultrastructural changes in unstable hemoglobin disease are: 1. The presence of inclusion bodies in the majority of the erythroblasts and reticulocytes; 2. the close association of inclusion bodies with clusters of iron-laden mitochondria; 3. Heinz-body formation within the nuclei of erythroblasts. The intranuclear inclusion bodies have essentially the same ultrastructure as the cytoplasmic precipitation of denatured hemoglobin compounds and 4. demarcation and autolysis of inclusion body containing cytoplasmic regions. Our ultrastructural findings suggest that inclusion body containing nuclei and digested Heinz-body containing cytoplasmic particles are extruded in the same way. It is assumed that the abnormal site and environment of hemoglobin breakdown are responsible for the abnormal metabolic pathway resulting in the appearance of pigmented dipyrrols which are excreted in the urine-S. R. H. CHANGES IN ENZYME ACTIVITY DURING RETICULOCYTE MATURATION AND RED CELL AGING. Ch. Bishop arid C. Van Cartel. From School of Medicine, State University of New York at Buffalo, N.Y. Haematologia 3:29-41, 1969. Activities of glucose-6-phosphate dehydrogenase (C-6-PD), 6-phosphogluconate dehydrogenase (6-PCD), hexokinase (HK), isocitrate dehydrogenase (lCD) and glutamic-oxalacetic transaminase (COT) were studied in rabbit and human red cells, separated according to density equilibrium by ultracentrifugation on bovine albumin For personal use only. on August 30, 2017. by guest www.bloodjournal.org From