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

The effect of serum from 32 AML patients on the normal human myeloid differentiation and the hexamethylene-bisacetamide induced granulocytic differentiation of HL-60 promyelocytic leukaemic cell line was studied. Nonadherent normal mononuclear marrow cells were cultured in vitro at a concentration of 5 x 10(5) cells/ml for 6 days with each of the 32 AML sera. Ten normal human AB sera were used as control. The results showed an inhibitory activity on both morphological and functional differentiation of normal human myeloid immature marrow cells by 29 out of the 32 AML sera tested. These 29 AML sera were added to cultures of HL-60 (2.5 x 10(5)/ml) leukaemia cell line which incorporated 2 mM hexamethylene-bisacetamide for 6 days. The results showed no significant inhibition of hexamethylene-bisacetamide induced granulocytic differentiation by any of the 29 AML sera. The efficacy of hexamethylene-bisacetamide in inducing differentiation in the presence of inhibitory factors suggests a possible role in the treatment of AML patients.

Some haemostatic parameters (AT III, alpha 2-AP, C1-INH, kallikrein, F.XII, fibrinogen, plasminogen, euglobulin lysis time, FDP and ethanol test) were studied in patients with deep (DVT) and superficial (SVT) venous thrombosis. The patients with DVT revealed significantly decreased AT III activity, increased alpha 2-AP, C1-INH activity, fibrinogen and FDP concentrations and prolongation of euglobulin lysis time. Ethanol gelation test was positive in 61% in DVT group. Plasminogen level was unchanged in patients with DVT. No significant changes in these parameters were found in SVT group. Only the ethanol gelation test was positive in 21% in this group. These results show a markedly expressed phenomenon of hypercoagulability in the group of patients with DVT and suggest that in the treatment different therapeutic procedures should be considered which influence these specific changes in these coagulation parameters.

Hyperviscosity syndromes can caused by both plasmatic and cellular factors. We have studied 20 patients affected by IgM gammopathy of different origin and 12 healthy subjects matched for sex and age, in order to evaluate the relation between paraprotein levels and plasma viscosity. We have observed a significant plasma viscosity increase only in 14 patients with monoclonal IgMk gammopathy. In the same patients was also evident an hyperviscosity syndrome. In the other 6 patients, with monoclonal IgM or polyclonal gammopathy and without clinical symptoms, plasma viscosity was only slightly increased. We have also observed a significant correlation between IgM and light chains (kappa, lambda) serum level and increased plasma viscosity. These results suggest that one can't consider all IgM gammopathies as cause of hyperviscosity syndrome.

Patients with thrombocythaemia due to myeloproliferative disorders (n = 21), with secondary thrombocytosis of various origin (n = 16), and a control group of healthy donors (n = 20) were investigated with respect to the aggregation behaviour and the total calcium content of blood platelets. The calcium content was significantly lower in both groups of patients as compared to controls (2 p less than 0.001). In 16 of 21 patients with myeloproliferative disorders platelet rich plasma did not respond to epinephrine (15 mumol/l), a concentration which induced at least weak aggregation in 14 of 16 patients with secondary thrombocytosis and also in healthy subjects. In patients with thrombocythaemia the mean extent of aggregation induced by epinephrine, collagen or adenosin diphosphate was significantly lower as compared to controls (2 p less than 0.001).

20 haemophilia patients known to have antibodies against F VIII for at least more than three years were treated on a regular base with 25 U/kg b.w. F VIII every other day. All 5 patients with previous maximal anti F VIII antibody levels between 5 and 60 BU/ml showed a decrease of antibody level and normal F VIII recovery within 1-2 months. From 12 patients with previous antibody levels above 60 BU/ml, 8 showed a disappearance of antibodies within 2-26 months. In 3 patients in whom no previous highest inhibitor level was known, one was treated successfully. Another group of 6 young patients in whom an inhibitor against F VIII had just (less than 3 months) developed, was treated with F VIII as soon as an inhibitor was detected. The dose infused was 25 U/kg b.w. F VIII twice weekly. In 5 patients this regimen was successful within 1-7 months. In the 6th patient the dosage was increased to every other day. One year after the beginning of therapy no inhibitor was detectable. So our results show that regular administration of F VIII in intermediate or low dose can lead to rapid disappearance of anti F VIII antibodies especially in patients with moderate inhibitor levels.

Paradoxical bleedings are complications occurring under replacement therapy in haemophiliacs by disturbancies of the primary haemostasis. They have been observed during treatment with factor-VIII- and prothrombin-complex concentrates of long duration and in high dosage. Clinical complications, for example delayed wound healing as well as spontaneous bleedings into the skin and from the mucous membranes, have been observed in one quarter of haemophiliacs under substitution therapy. In one third of these patients pathological parameters of primary haemostasis (prolonged bleeding time, reduced retention, retraction, ADP- and collagen-induced aggregation and the platelet factor 3 release) were found out. The following mechanisms or substances may be the cause for these disturbancies: 1. fibrinogen and factor-VIII split products 2. high content of proteins predominantly fibrinogen and factor-VIII-related antigen 3. antigen-antibody reactions 4. development of inhibitors against the Willebrand factor. For treatment of the paradoxical bleedings freshly prepared cryoprecipitate, prednison and Etamsylatum have been used.

Some oxidative indexes and the level of reduced glutathione have been determined on red blood cells stored for transfusion use. Conjugated dienes show a concentration which increases until around the third week of storage and then comes back to lower values. On the contrary malonyldialdeyde shows a decreasing level until the third week and then rises rapidly to very high values. Reduced glutathione rises until about the end of the second week and then falls to very low values. Free haemoglobin continuously rises through the whole period of storage; from these data it is evident that a storage time not exceeding 21 days gives the best transfusion results.

The human neutrophils induce cytotoxic effects on mammalian tumor cells. Hence it may be expected that intracellular enzymatic deficiencies of neutrophils may represent another cancer risk factor. In 205 patients with various malignancies the neutrophil beta-glucuronidase activity has been determined using a semiquantitative cytochemical method. A statistically significant deficiency of this enzyme in neutrophils has been observed in patients with precancerous states of the larynx, cancer of the larynx after radiotherapy and patients with cancer of large intestine. Patients with cancer of the lung and cancer of the stomach showed no changes with that regard whereas those with cancer of the breast demonstrated an increased enzyme activity.

The binding of five fluorescein-labelled lectins: peanut agglutinin (PNA), lentil agglutinin (LEN), soybean agglutinin (SBA), wheat germ agglutinin (WGA) and asparagus pea agglutinin (ASP) to human B-cell chronic lymphocytic leukaemia (B-CLL) and B lymphocytes of normal donors was studied. The specificity of the fluorescence was demonstrated by inhibition with appropriate saccharides. The proportion of B cells was estimated using anti-B cell monoclonal antibody. Both leukaemic and normal B cells showed the binding ability of all except of one (ASP) studied lectins. We have found the differences in surface carbohydrate patterns between B-CLL and normal B lymphocytes. B-CLL cells showed the considerably lower ability to bind SBA and slightly higher expression of PNA and LEN receptors in comparison to normal B cells. The analysis of WGA binding allowed for recognizing two groups of CLL patients: one with high and the second one with low WGA receptor expression. The double marker studies revealed that B cells could simultaneously react with anti-B cell monoclonal antibody and fluorochrome labelled lectins.

The ability of plasma from ITP patients (before and after splenectomy) to support the growth of megakaryocyte progenitors was compared with that from healthy subjects. Plasma Factor Index-Megakaryocyte PFI-Mk (ITP) which expressed resultant colony growth was significantly lower before splenectomy, but it normalized after splenectomy. (PFI-Mk) (ITP) did not relate neither to megakaryocyte nor to platelet counts. A positive correlation has been observed between megakaryocyte and platelet numbers in healthy subjects and in ITP patients after splenectomy, but not before splenectomy. The proportion of immature megakaryocytes was markedly higher in ITP marrow before splenectomy. This study indicates, that in ITP apart from antibodies directed to platelets and megakarocytes a low plasma stimulatory activity affected megakaryocytopoiesis.