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Biomedical subjects

K Rák

Publications and source records attributed to K Rák.

At least 37 records · Page 2Linked to original sources

[Hypocholesterolemia and other lipoprotein disorders in myelofibrosis].

In the recent years more and more data suggest a significant relationship between malignant diseases and cholesterol, respectively lipoprotein metabolism. It is a significant decrease of cholesterol in primary myelofibrosis (agnogenic myeloid metaplasia) and in secondary myelofibrosis. Similarly, the HDL and LDL cholesterol levels are also decreased in these diseases. On the contrary, the triglyceride level is significantly higher in these groups, 41 patients with agnogenic myeloid metaplasia and 16 patients with myelofibrosis following polycythaemia vera were examined. Decrease of serum cholesterol level was significant: 3.7 and 3.3 mmol/l and 5.2 mmol/l in 76 healthy controls, as the mean values.

Aged↗

[Eosinophilic leukemia: a rare form of Philadelphia chromosome negative chronic myeloid leukemia?].

Eosinophil leukaemia is a rare and poorly defined entity characterized by neoplastic proliferation of eosinophil cell line. This form of the hypereosinophilic state is considered to be a variant form of CML, although as a diseases entity is not generally accepted. A history of a patients is reported, whose clinical course is thought to fulfill the requirements of eosinophil leukaemia. On the basis of the initial results (pathological lymphogram, eosinophilia, Ph-negativity) lymphogranulomatosis was suspected and explorative laparotomy was performed. However, only marked eosinophilic infiltration of the spleen was detected. After splenectomy his disease was stable without treatment for six months when his leukocytosis and eosinophilia increased. Despite the administration of hydroxyurea the leukocyte count exceeded 100 x 10(9)/l (eosinophil cells 70%), and the bone marrow revealed massive (80%) eosinophilic infiltration. Neither Ph-chromosome, nor cabl and bcr gen rearrangement were demonstrated, but the expression and amplification of c-myc oncogene indicated disease progression. Interferon therapy produced long-term clinical and haematological improvement, but blastic transformation was developed in the second year of his disease. Autopsy showed multiple organ involvement characteristic of CML, but no marked eosinophilic infiltration was found. The feature of this case suggest that eosinophil leukaemia might represent an uncommon form of Ph-negative CML.

Adult↗

[Current possibilities in the treatment of high-malignity non-Hodgkin lymphoma, with special reference to autologous hematopoietic stem cell transplantation].

The present combined radiochemotherapy results in a complete remission in 50-60 percent of patients suffering from high-grade non-Hodgkin's lymphoma but only about half of them are cured. The basic regimen for chemotherapy is still the CHOP. There are known "salvage"-protocols, among them the CEPP-regimen, for patients who relapsed. For the still chemosensitive patients a newer complex potentially curative treatment procedure is available. Stem cells harvested during the first or second, in certain cases in a subsequent good remission from the bone marrow or even more from the peripheral blood may be later useful in protecting patients from a severe myelodepression or mostly as a "haemopoietic rescue" in restoration of the bone marrow function after myeloablative conditioning. The present treatment strategy in progressive disorders is mainly based on the complementary effect of intensive radiochemotherapy, autologous stem-cell transplantation and the rational use of cytokines, mostly colony-stimulating factors.

Antineoplastic Combined Chemotherapy Protocols↗

[Correlation between thrombocytes and fibrinolysis].

After a concise review of the fibrinolysis modifying effect of platelets, a simple, reliable and reproducible in vitro microplate clot-lysis method is described which is able to demonstrate the lytic resistance of platelet rich clots. The same method proved to be suitable to show the enhanced lysis in platelet rich lytic environment, caused probably by plasminogen attached to platelet surface. The results discussed here suggest that these effects need the presence of intact platelets.

Adult↗

[Correlation between adhesive factors of primary hemostasis and liver fibrogenesis in alcoholic live cirrhosis].

Colchicine administration is able to reduce collagen production and the fibrogenetic process in alcoholic liver diseases in the same time. The decrease of the serum lipid peroxidation capacity along with type III procollagen propeptide level was associated with the simultaneous reduction of plasmatic von Willebrand factor, fibronectin and beta-thromboglobulin levels. This observation suggest a connection between the regulation of primary haemostasis and liver fibrogenesis.

Colchicine↗

Antiretroviral immune response and plasma interferon in different phases of chronic granulocytic leukemia.

Forty patients with chronic granulocytic leukemia (CGL) were tested for antibodies and lymphocytes reacting with gibbon ape leukemia virus (GaLV) and baboon endogenous virus (BaEV) antigens as well as for plasma interferon levels. Antibodies reacting with envelope antigens of GaLV and BaEV were found frequently and in high titers in patients with the quiescent phase of CGL but rarely and in low titers in the accelerated and blastic phase of the disease. Results of radioimmunoprecipitation studies were in concordance with those obtained in virus neutralization experiments. Cellular and humoral cytotoxic activity of blood plasma and lymphocyte samples against autologous tumor cells showed a similar phase-specific distribution. Most of these activities could be blocked by GaLV and BaEV gp70 antigens. Elevated plasma interferon (IFN)-alpha levels were found in the quiescent and accelerated phase of CGL, whereas no significant differences could be detected between IFN levels of patients with the blastic crisis of CGL and those of the control persons. Follow up studies of four patients confirmed this stage-specific distribution of antiretroviral immune and interferon response.

Animals↗

[Primary hemostasis studies in Conn syndrome].

The various components of haemostasis seems to be affected by the high cortisol level in Cushing's disease. Increase in coagulation factor levels, decreased fibrinolysis, high von Willebrand factor plasmatic levels along with easy bruising due to vascular damage have been described. In this report a similar but less accentuated elevation of von Willebrand factor levels are documented in aldosterone producing adrenocortical adenoma, and in the same time enhanced platelet activation was also found. The primary haemostatic alterations correlated well with aldosterone levels and subsided significantly after the removal of the ademonas. Further studies seem to be desirable to analyse and elucidate haemostatic functions in Conn's syndrome.

Adult↗

[Secondary acute leukemia in Hodgkin's disease].

Two secondary acute leukaemias out of 166 patients with Hodgkin's disease were detected. At a young female patient treated only with chemotherapy the acute leukaemia developed 39 months following the diagnosis of Hodgkin's disease. Phenotype of leukaemic blast cells could not be determined exactly. After a short complete remission (1,5 month) the patient died because of the progression of her leukaemic process. The other young male patient treated with radio- and chemotherapy had six years disease free interval between the diagnosis of two malignant diseases. The combined cytostatic treatment of his acute lymphoblastic leukaemia resulted complete remission that lasted for one year but later the progression of leukaemia caused death. In connection with these cases the authors reviewed the current questions of treatment related acute leukaemias in Hodgkin's disease.

Adolescent↗

[Successful electroconvulsive treatment of a schizophrenic patient suffering from severe hemophilia A].

Experiences obtained with the electroconvulsive treatment of a schizophrenic patient suffering from severe haemophilia A are reported. No haemorrhagic complications were observed. According to our experiences electroconvulsive therapy can be applied also in severe haemophilia A if satisfactory replacement therapy can be ensured. Similar case has not been found in the literature.

Adult↗

HTLV-related markers in a Hungarian patient with adult T-cell leukemia.

Monoclonal integration of DNA sequences related to, but not identical to HTLV-I provirus was detected in the peripheral blood lymphocytes of a Hungarian male suffering from ATL. The patient and his parents showed serological cross-reactivity with both HTLV-I and HTLV-II group-specific antigens. Restriction enzyme analysis with EcoRI, PstI, BamHI, HindIII and SacI revealed structural similarity of the provirus integrated in the DNA of ATL cells to HTLV-I but not to HTLV-II. Data suggest that this provirus and HTLV-I are similar to each other along gag and pol regions, but they are different in the env region.

Adolescent↗

[Primary hemostasis in mixed connective tissue disease].

Willebrand-factor antigen level and structure analysis, ristomycin-cofactor assay, beta-thromboglobulin and thromboxane metabolite estimations were performed in 22 patients with mixed connective tissue disease. High levels of Willebrand factor antigen and activity were detected in the presence of thrombocytopenia, previous thrombotic events, pulmonary vascular lesions and in the presence of circulating antiendothelial antibodies. Increased platelet activation was documented also in antibody positive cases and in thrombocytopenia. The alterations of endothelial and platelet functions may play important role in the development of vascular complications of mixed connective tissue disease.

Adult↗

[Successful treatment of severe hemorrhage by using thrombocyte suspension with cryoprecipitate in type III Willebrand disease].

A 24-year-old woman with severe haemorrhagic complication due to type III von Willebrand's disease is reported, as the first such case in Hungary. Analysis of seven family members affected by the type III mutant gene was compatible with an autosomal recessive mode of inheritance. Severe, iatrogenic epistaxis of the patient was not improved by cryoprecipitate and DDAVP. Platelet suspension together with cryoprecipitate immediately stopped bleeding. Simultaneous application of cellulare (platelet suspension) and plasmatic (cryoprecipitate) Willebrand protein is suggested for patients with severe (type III) von Willebrand's disease.

Adult↗

[Successful cyclosporin therapy of acquired hemophilia caused by factor VIII (VIII:C) inhibiting antibody].

A 47-year-old woman is reported who had a life-threatening, subarachnoideal bleeding due to the spontaneous development of factor VIII:C inhibitor. Cyclosporin combined with prednisone allowed a full recovery and the elimination of antibody even when other therapeutic facilities failed to be effective. Actual problems of treatment of factor VIII inhibitors are briefly surveyed.

Antibodies↗

[The myelodysplastic syndrome].

Clinical data of 70 patients, treated and observed with myelodysplastic syndrome between 1977 and 1989 were analysed. Two-thirds of the patients belonged to the elder age-group and a mild female predominance was registered. With the application of complex cytochemical-histological and cytogenetical methods, correct diagnosis could be established. The clinical material included patients from different morphologic subtypes: 19 with refractory anaemia (with a longer course of the illness). 20 with sideroblastic anaemia, 26 with chronic myelomonocytic leukaemia and the remaining 5 with refractory anaemia with excess of blasts (a more progressive type of the myelodysplastic syndrome, with a short duration). The mean survival of all patients were 42 months. 45 (69%) died during this period and 12 (18.5%) among them in acute myelogenous leukaemia (mean survival: 16 month). Megakaryoblastic leukaemic transformation was observed in three patients with sideroblastic refractory anaemia. Haemorrhage and infection-sepsis, due to thrombocytopenia and/or granulocytopenia, was fatal in 30 cases. In the treatment of the myelodysplastic syndrome an appropriate supportive therapy (blood transfusion, antibiotics) has a decisive importance. A more aggressive treatment with cytostatic drugs is suggested in the progressive form of the disease of younger patients and in patients with overt acute leukaemia.

Adult↗

[Thrombotic changes in hemostasis following streptokinase therapy in myocardial infarct].

Thrombolytic treatment of acute myocardial infarction proved to be able to restore infarct artery patency and to decrease hospital mortality. The number of bleeding complications have remained at an acceptably low level, however some thromboembolic complications occurring during the first week following thrombolytic therapy have been recently observed. Signs of increased in vivo platelet activation (by measuring beta-thromboglobulin and thromboxane metabolite levels) and endothelial damage (Willebrand-factor estimations) could have been detected in our patients treated with brief high dose intravenous streptokinase, altogether with diminished antithrombin III and protein C antigen levels and activity. Intravenous streptokinase treatment of acute myocardial infarction might be able to cause thrombotic haemostatic alterations, which require meticulous haemostasis monitoring and early, correct antithrombiotic therapy.

Blood Coagulation↗