Long-standing prophylactic therapy vs. episodic treatment in young people with severe haemophilia: a comparison of age-matched Danish and Russian patients.

Two distinctly different substitution principles are commonly used in haemophilia: treatment at bleeding episodes only referred to as on-demand treatment, and prophylactic factor administration. The aim of the cross-sectional study which was undertaken in young patients suffering severe haemophilia A was to challenge our hypothesis that on-demand treatment is inferior to prophylactic substitution in prevention of chronic joint disease at young age. The method involved an investigation of 40 patients from Russia (n = 27) and Denmark (n = 13) born between 1975 and 1990 with no history of inhibitors; Russian patients had exclusively received factor VIII on demand, while Danish patients were managed with prophylactic treatment during a mean period of 16 years since median age of 5 years. The study endpoints were clinical joint scores, Quality of Life scores and functional independence scores. Matched by identical age (±1 year) 13 Danish and 13 Russian patients were compared, while 14 age similar Russian patients served as controls. Demographic data among all groups were quite comparable. The results are that Russian patients presented with clinical joint scores at 27 ± 8.5 (mean ± SD) while matched Danish counterparts scored 3.8 ± 5.3 (mean ± SD), differences being highly significant. The number of joint bleeds in recent 5 years were 199.5 ± 135 (mean ± SD) vs. 8.1 ± 8.7 (mean ± SD). Likewise, Quality of Life and functional independence scores were significantly higher in patients on prophylaxis as compared to on-demand treatment. In conclusion, the study outcomes confirmed our hypothesis. Longer term prophylactic factor administration during childhood and adolescence prevents joint destruction.

[Determination of the electrophoretic mobility of peripheral red blood cells from healthy individuals, by using ethylene-2-amine-4-chloroacetic acid as a preservative].

It is now known that the electric charge of a surface cellular membrane is a rather characteristic value for each type of a cell. Cellular electrophoresis may be used to study the electrokinetic properties of red blood cells in each specific case, thereby obtaining indirect information on the state of a surface membrane as the electrophoretic mobility (EPM) rate is directly proportional to the value of a cellular electric charge. The study was undertaken to compare the EPM rates of peripheral red blood cells from donors, by employing two different preservatives: EDTCHA and sodium citrate. No statistically significant differences were found in the groups under study and these parameters were comparable.

[Platelet adenosine desaminase in various hematological diseases]. / Adenozindezaminaza trombotsitov krovi pri razlichnykh gematologicheskikh zabolevaniiakh.

Activity of adenosine deaminase (EC 3.5.4.4) was studied in thrombocytes of donors and patients with various hematological diseases. The enzymatic activity was decreased in acute leukemia, chronic myeloleukemia, chronic leukemia and blast transformation myeloma, microspherocytic and hypoplastic anemias. Variable level of the activity was observed in chronic lympholeukemia and non-Hodgkin disease. In all the diseases studied functions of thrombocytes were altered after treatment with various aggregating agents (ADP, thrombin, collagen, adrenaline, ristomycin).

[Thrombocyte purine nucleoside phosphorylase in various blood diseases]. / Purinnukleozidfosforilaza trombotsitov pri razlichnykh zabolevaniiakh krovi.

Activity of purine nucleoside phosphorylase (EC 2.4.2.1) was estimated in thrombocytes of donors and of patients with various hematological diseases. The enzymatic activity was decreased in chronic lymphoid leukosis chronic myeloleukemia, chronic myeloleukemia and blast transformation, acute lymphoblast and myeloblast leukemia, myeloma. Dissimilar level of purine nucleoside phosphorylase activity was found in non-Hodgkin disease. The activity was only slightly increased in Waldenström's macroglobulinemia; it was similar to normal level in hypoplastic and autoimmune hemolytic anemias. A decrease in the enzymatic activity was observed in thrombocytes of the patients with hereditary forms of hemorrhagic diatheses (hemophilia A, Willebrand disease). Normal level of the enzymatic activity was noted in Glanzmann thrombasthenia.

[Adenase inhibitor in thrombocytes of healthy humans]. / Ingibitor adenazy trombotsitov zdorovykh liudei.

An inhibitor of adenase, which is absent in leukemic patients, was studied in thrombocytes of healthy persons. The inhibitor, localized in the granular fraction, was isolated by ultracentrifugation in sucrose gradient.

[Some indices of the fibrinolytic system of the blood in patients with acute leukemia]. / Nekotorye pokazateli fibrinoliticheskoi sistemy krovi u bol'nykh ostrym leikozom

An attempt is made to ascertain the mechanism of increased fibrinolytic activity in patients suffering from acute leukemia. Investigations confirmed a possibility of both the primary and the secondary character of hyperfibrinolysis which required the choice of rational therapeutic tactics in each individual case.