Bilten za hematologiju i transfuziju最新文献

The synthesis of fetal hemoglobin was investigated in in vitro cultures of erythroid precursors isolated from peripheral blood of normal individuals, newborns and of subjects with different hemoglobinopathies. Synthesis of hemoglobin was assessed by 35s-methionine labeling of cultures and measurement of the radioactivity incorporated into the hemoglobins A2, A, F and S isolated by column chromatography on DE 52 cellulose. The erythroid precursors from most of the studied individuals cultured in in vitro system responded with synthesized an average of 15% of Hb F while cultures from newborns produced an average of 60% of Hb F in comparison of 73% of Hb F in peripheral blood of the same newborns. Erythroid precusors from subjects heterozygotes for beta-thalassemia, heterozygotes for HPFH, and homozygotes for Hb S produced an average of 20%, 43% and 30% of Hb F, respectively, in comparison of 7%, 14% and 9% of Hb F, respectively, present in the RBC of the same individuals. These data support the previously published results (6-11) that erythroid bursts in culture reactivate the structural genes for the gamma chain synthesis.

The action and toxic effects of Actinomycin D are discussed. Emphasized is the appearance of hepatic disfunction, that develops especially when the drug is applied during the first six months after hepatic irradiation. Two children with malignant tumor are demonstrated. In both a temporary hepatic lesion appeared after treatment with Actinomycin D.

A group of 30 preterm babies with no evidence of infections was investigated. They were separated in 3 groups according to the gestational age. In the first group, which included babies 28 to 32 weeks of gestational age, haptoglobin was detected in 5, but in 3, levels were under 25 mg/dl. In the second group, which consisted of babies of 33 to 36 weeks of gestational age, haptoglobin was detected in 6 infants, with levels less than 25 mg/dl in 3. Finally, in the group of babies of 37 to 40 weeks, haptoglobin was presented in 7. Haptoglobin was detected in 18 infants, which means in 60% of patients. Levels of haptoglobin were lower in babies of lower gestational age. Values obtained for haptoglobin levels in this study will be used in diagnostic purposes, for comparation with levels obtained in various pathological conditions.

In the previous paper published in the Bulletin for Hematology and Blood Transfusion we described the congenital deficit of Hageman factor (HF) with the basic findings. Now we provide addilioval laboratory -diagnostical tests in order to confirm definitively that our patients have the deficit of Factor XII, and not of some other factors of the contact coagulation phase as: Fletcher, Fitzgerald, Williams and Flaujeac. On the other hand, in order to enlight the laboratory-diagnostical problems which one can face in solving of these cases, we have reviewed the basic biochemical characteristics of the contact factors and the mechanism of the beginning of the internal pathway of blood coagulation.

Acute leukemias in infants, including the congenital and neonatal leukemia, present a number of unfavourable features. Age of the infant, hyperleukocytosis, outstanding organomegaly, early onset of the CNS leukemia are some of the factors causing this group of acute leukemias of childhood to be those with highest risk. The poor prognosis of the illness is further worsened by frequent rejection of cytotoxic therapy, often failure in inducing remission and its shortness. Two patients with acute lymphoblastic leukemia, aged 4 months, are presented. Clinical the hematologic remission was achieved in both by the application of the current therapeutic methods. Recurrence and CNS leukemia appearing 5 months after the remission resulted in death of one patient. The second patient also developed CNS leukemia 5 months after remission. It was treated and another remission was achieved, but the child died due to interstitial pneumonia.

It has been analised cases of toxic methemoglobinemia in newborns and sucklings on patients of children department city hospital Sombor in period from 1968 to 1979. In all patients methemoglobinemia was caused with wellwater containing nitrates. Originate of this state depends on: growth of child because in first few month of life they have immature methemoglobin reductase in erythrocyte, hypovitaminosis C and gastrointestinal disfunction. Diagnosis was based on anamnestical data that was used water from unhygenic well, on cyanosis of various intensity and that have disappeared during the treatment with vitamin C and not give any recurrence later. In last years number of such cases is decreasing because of better suply with proper drinking water. Further decrease can be achieved with health education and prophylactic peroral consumption of vitamin C in predisposing regions.

A homozygot carrier of a congenital nuclear anomaly of blood cells/Pelger-Huet's anomaly: abnormal segmentation of granulocytic leukocytes) is described. The morphologic analysis of blood samples in 4 family members showed similar Pelger-Huet's anomalies. The phagocytic activity, the "killing test" and the inhibition test of leucocyte migration of the patients neutrophils were in normal ranges.

Isolated and highly purified myeloma IgD DEK, STA and SAR were subjected to isoelectric focusing in thin layer of polyacrylamide gel using equipment and PAG plate 3, 5-10 from LKB. Although homogeneous in electrophoresis on cellulose acetate folien, immunoelectrophoresis and DISC PAA gel electrophoresis analysed IgD showed high isoelectric heterogeneity. They formed isoelectric spectra with 24-27 pl zonnes ower the pH range 5,4-9,3. Based on densitometric analysis of gel stripps zonnes with a small protein content were excluded from calculation of the real isoelectrical range. According to that manipulation isoelectrical range was determined as pH 6-8. Heterogeneity of isolated myeloma IgD may be due to the post-synthetic transformation of molecules in vivo as well to degradation and/or aggregation of IgD in vitro during the preparation of samples for isoelectrofocusing. However, myeloma IgD are in fact more heterogeneous in isoelectrofocusing than myeloma immunoglobulins of other classes.

A case of a rare congenital combined deficiency of factor V (14%) and factor VIII (30%) is described for the first time in our literature. Nine years old girl was admitted to hospital because of nightly bleedings from hyperplastic and inflamed gums. Clinical picture was mild what is in accordance with the results of coagulation studies. Under substitution therapy tooth extraction passed without complications.

The authors observed 70 patients with acute leukemia treated by polychemotherapy. In 2 patients the signs of severe liver damage were found. The occurrence, course and results of laboratory tests suggested Purinethol predominantly as the cause of development of these alterations. The complication did not occur frequently in our patients. The changes regressed in a satisfactory way after the withdrawal of the hepatotoxic cytostatic from the therapy.