Folia haematologica (Leipzig, Germany : 1928)最新文献

The light and electronmicroscopic representation of non-haemiron in the bone-marrow provides the unique opportunity of extensively evaluating the iron metabolism. In the bone-marrow, macrophages represent the physiological place of iron storage. The iron in the cytoplasma is stored in them in the form of free ferritin molecules and lysomally as aggregated ferritin and/or haemosiderin in siderosomes. In an equal iron balance and unimpaired internal iron exchange only erythroblasts (sideroblasts) and erythrocytes (siderocytes) of the bone-marrow besides macrophages possess siderosomes. In addition to this physiological or orthotopic iron storage a heterotopic iron storage can be observed under pathological conditions, particularly with iron overloading of the organism, in the endothelial cells of sinusoids and plasma cells. In detail, the patterns of iron storage in the bone-marrow are described in the different stages of iron deficiency, disturbance of iron utilization in chronically inflammatory processes or tumour diseases, condition after intravenous iron administration, transfusion siderosis, hereditary haemochromatosis and sideroblastic anaemia.

The retrospective analysis has concerned 323 patients with acute nonlymphocytic leukaemia (ANLL). The comparable patients groups were treated since 1981 according to protocols used by the Polish Acute Leukaemia Group (induction; modified TAD or Adriamycin plus Ara-C, maintenance; rotatingly changed polychemotherapy for 3 years). The prognostic value for achieving complete remission (CR) and survival of 67 pre-treatment factors (42 quantitative and 25 qualitative) was evaluated. The most important 9 parameters were scored according to the prognostic value as follows: age, proportion of blasts in bone marrow, blast count in peripheral blood, morphological subtype, percentage of granulocytes in bone marrow, proportion of blasts with CD-15 antigen, thrombocyte count, spleen/liver enlargement, protein concentration in cerebro-spinal fluid. The scoring system has been elaborated allowing selection of ANLL patients to standard risk group and a high risk group.

The effect of supernatant of thymus-cell conditioned medium (TCCM) on blood element recovery in peripheral blood was followed in mice exposed to a single whole body dose of 5.8 Gy of gamma radiation. As follows from our results. TCCM administered 18 h before irradiation accelerated the recovery of the reticulocyte and, in part, thrombocyte and granulocyte counts. However, no effect on the rate of lymphocyte recovery was found.

With Prussian blue reaction nonhaemoglobin iron in the erythroblasts is demonstrable. Three pathological sideroblast types are recorded separately: abnormal intermediate type I and II sideroblasts and ring sideroblasts, representing increasing levels of sideroachrestic disturbance. This permits the classification of sideroachrestic disturbances into four degrees of seriousness. The frequency of a sideroachrestic disturbance in 47 untreated patients with acute myeloid leukaemia was 87%. Among 11 patients with preleukaemic condition, 8 had a disturbance of iron utilisation. In both preleukaemia and leukaemia mainly intermediate sideroblasts were present. All patients with preleukaemia developed leukaemia within 1-20 months. In the course of preleukaemic condition a slight increase of iron misutilisation was obvious when terminating in overt leukaemia. This could be of prognostic importance. After treatment, pathological sideroblasts disappeared only in 2 out of 15 patients with complete remission. There was no correlation between effect of therapy and course of iron misutilisation.

The arthrosonography is a new depicting procedure for detection of destructing joint processes in haemophilia. A direct evaluation of the cartilage, of the border between cartilage and bone, of the synovial membrane with proliferative processes as well as the differentiation between soft-tissue bleeding and haemarthros are possible. An essential advantage is given by a combined morphological and functional joint evaluation. The diagnosis of joint instabilities can be recognized easier by sonography than by X-ray. Clinical findings are completed in an excellent manner and lesions of bone and cartilage are detected earlier by arthrosonography.

The principle of the FEIBactivity hadn't been defined in a sufficient manner up to now. Probably the factor VII or factor VIIa are playing a key role. According to own experiments the factor VII activity increases considerably in activated prothrombincomplex concentrates together with the quotient factor VIIa/VII. Factor VII should be administered in his activated form for bypassing. Fractions with limitation of the activation process to factor VII are of a low thrombogenicity. Among activated coagulation factors factor VIIa persists longest in the circulation and a bypass efficacy can be provoked only by this.

The mechanism of the bypass activity of prothrombin complex preparatives for treatment of haemophiliacs with circulating anticoagulants remains unclear up to now. The following parameters had been tested with preparatives produced in a different manner (absorption by DEAE-Sephadex A 50, Molselekt A 50, tricalcium phosphate or aluminium hydroxide): Coagulation factors II, VII, IX, X and XII, activated factors VIIa and Xa as well as the FEIB activity. The bypass activity is strongly correlated to the factor VIIa content of the preparatives, however. These results agree with the statements by HEDNER and KISIEL, which had used a factor VII a concentrate for treatment of haemophilia complicated by circulating anticoagulants with success. According to farther investigations factor VII is correlated with the lipid content of the starting plasma. This knowledge is important for optimizing the production procedure of prothrombin complex preparatives with bypassing activity.

Determinations of thrombin/antithrombin III complex (TAT) in human plasma with the new enzyme immunoassay (Enzygnost-TAT) were performed. The study group consisted of 36 patients aged 36-79 years suffering from peripheral obliterative arterial disease (POAD) comparing to control group. In patients the elevation of TAT level was detected. The analysis of increased TAT level in the relation to clinical atherosclerotic complications, the kind of treatment, the stage of POAD, the age of patients and the period after operation was done. The highest TAT complex seemed to be correlated with the advance of atherosclerotic process, with additional diseases such as arterial hypertension, diabetes mellitus and performed operation. The "Enzygnost-TAT" assay can be useful for the detection of prethrombotic states without clinical symptoms of hypercoagulability and can be applied for clinical diagnosis.

Especially in AML but also in ALL a dose reduction during the induction therapy effected distinctly both a diminution of the CR rate and a shortening of the LFS. For these reason reduced treated patients are to exclude from final analysis of study in order to obtain a objective comparison of the four postremission treatment modalities. There was no difference concerning treatment related mortality between "correct" and "reduced" induction therapy.