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

30 rabbits received an infusion of lipopolysaccharide B (75 micrograms/kg.h) over 4 hours (groups E, EI, EA; n = 10 each). Saline was given to a control group (C; n = 8). In group EI, prostacyclin (PGI2; 500 ng/kg.min) was given simultaneously to endotoxin. Into group EA animals, aspirin (20 mg/kg) was injected before the endotoxin infusion was started. PGI2 and aspirin both improved survival of animals (6/10 each vs. 2/10 in group E). The drop of platelet counts was significantly reduced by PGI2, while leukocyte depletion was similar in all endotoxin groups. PGI2 preserved the functional capacity of platelets as indicated by collagen stimulated aggregation and thromboxane formation. PGI2 but not aspirin significantly reduced renal fibrin deposition.

The use of thickness of protein layers adjacent to the surface of each erythrocyte is discussed as a diagnostic test. The given method of studying such layers is based on electrophoretic measurements which make it possible to evaluate the structure, composition and thickness of these layers. It has been shown that the normalization of erythrocyte electrophoretic mobility of stored or pathologic blood flowing through an active-carbon column may be explained by a full or partial removal of adsorption protein layers and intermicellar liquid contained it.

The object of this study was to determine whether the "in vitro" parameters of medullary and blood granulopoiesis in patients with MDS, furnish information of either prognostic or diagnostic value. This study covered 94 patients with MDS. All patients were studied at the onset of disease. In order to identify the factors related to patients' survival, Cox Multiple Regression analysis was performed by the BMD P2L program. When analyzing by means of actuarial curves the survival probability of patients with benign development versus those of malignant development (those who developed ANLL), the significance between both groups was p = 0.0001. Different variables of patients included in this study were analyzed and all showed great significances. Fab: p = 0.0022, disease evolution: p = 0.0001 and presence of blastic aggregates: p = 0.0011. Cox's regression analysis revealed that the only predictable survival variable is the presence of blastic colonies and/or clusters. Accordingly, two groups were constructed: favourable and unfavourable. In the favourable group, 40% of the patients belonged to the RA group, while in the unfavourable group, 55% belonged to the RAEB group. This study shows the validity of the elaboration of prognostic groups in MDS according to the presence of blastic colonies and/or clusters in CFUGM medullary and/or peripheral cultures. The "in vitro" myeloid progenitors culture techniques may therefore be advantageously applied in these disorders for formulating a diagnosis and predicting the patient's short term evolution.

174 serum ferritin assays in 121 patients with various haemolytic disorders have been performed. The mean serum ferritin levels were significantly increased in all these disorders in contrast to healthy controls. The highest serum ferritin levels were found in pyruvate kinase (PK) deficiency, moderate increase was observed in hereditary sphaerocytosis (HS) and in autoimmune haemolytic anaemia (AIHA) with massive haemolysis and in glucose-6-phosphate dehydrogenase (G-6-PD) deficiency. Mild elevation of serum ferritin levels was depicted in paroxysmal nocturnal haemoglobinuria (PNH), in beta thalassaemia minor and in other types of haemoglobinopathies. The range of values was associated with a degree of haemolysis and its relation to duration of the disease was not apparent in most cases. Highly significant differences between serum ferritin levels in splenectomized and non-splenectomized patients with HS and between serum ferritin levels in patients with AIHA with massive haemolysis or in remission were found. As compared to normal controls, significant increase of serum ferritin levels was observed even in patients with AIHA in remission or in splenectomized patients with HS. In two patients with PK deficiency the levels exceeding 2,000 micrograms/l indicated manifest iron overload. A reliability of serum ferritin assay as an index of iron stores in haemolytic disorders has been discussed.

Acidified glycerol lysis test (AGLT) was found to be an appropriate technique for detection of sphaerocytes in blood particularly in hereditary sphaerocytosis. The incubation of blood for 5 hours at 37 degrees C prior to measurement exerts positive effect on the reliability of the test and may further suggest the type of haemolytic disorder.

The effect of heated serum at a concentration of 10% in culture on the in vitro growth of confluent Balb/c 3T3 cells was studied in nine patients with Idiopathic Myelofibrosis and ten normal subjects. Patients showed significant increase in the mitogenic activity in comparison with normals. The growth factors conceivably implied for the observed effect are discussed. Particular attention is paid to Platelet-Derived Growth Factor from which serum mitogenic activity is primarily derived and is thought to take part in the genesis of bone marrow fibrosis.

We have measured the effect of normal B lymphocytes on more primitive granulocyte progenitors (CFU-dG) clonal growth in double-diffusion chamber culture in vivo. It was found that pokeweed mitogen (PWM) stimulated B cells produce a growth-promoting activity which augments the CFU-dG--derived myeloid colony formation in a dose-dependent fashion. All colonies formed under the experimental conditions were composed exclusively of granulocytes at different stages of maturation. Unstimulated B lymphocytes did not effect the CFU-dG clonal proliferation.

There is an evaluation of routine examination of the blood film for the recognition of Thalassaemia (Th.). The results of 100 blood films from each group--Th. major, Th. minor, healthy persons (centre for Thalassaemia "Bishop Makarios"/Nikosia--Cypros)-were analyzed. MCV and MCH were most useful for the recognition of Th. minor. The constellation of increased red blood cells and normal hemoglobin seems to be typical for Th. minor. Haemoglobin and haematocrit are not suited because they were widely normal. Target cells also are not sufficient for screening--they were present only in 10% of Th. minor. To the contrary most values were clearly decreased in Th. major, and target cells were present here in almost 90%.

In a brief survey four groups of determining methods are reported for the purpose of monitoring the heparin therapy, with their advantages and disadvantages being assessed simultaneously. Emphasis is placed on the heterogeneity of heparin with approximately 120 fractions which are the cause of different manners of response and an impediment to the development of a rapid, reliable and economically advantageous method. Finally, all heparin factors in the plasma are enumerated and their mechanisms of action on individual coagulation factors are referred to.

The joint condition in 98 children and adolescents with severe or moderate types of haemophilia participating in a summer camp between 1987 and 1989 had been analyzed. This was done by the centers' report and on the other hand with testing the patients by aid of the score system recommended by the World Federation of Hemophilia. According to the centers' reports more than 50% of all patients seem to be free of joint contractures. But according to the more sensitive score system more patients are affected by discrete symptoms of the chronic arthropathy. There had been discovered big differences between the centers and an increase of the patient's score by age. Not seldom an impairment of the findings within a single year was recognized. The joint problems in haemophiliacs are solved by the prophylactic treatment in an incomplete manner only. Lacks in the sophisticated evaluation of joints, with the management of prophylactic treatment as well as adjustment to prevention lead to avoidable damage crippling the patient on later age.