Vol 15, No 3 (2016)

Autoimmune haemolytic anaemia (AIHA) is a rare disease characterized by formation of red blood cell autoantibodies. A precise classification of AIHA types is very important. There are warm (w-AIHA) and cold (c-AIHA) AIHA with cold haemolysins, and drug-induced AIHA. Each type has different serological characteristics of autoantibodies, which determines the mechanism of red blood cell destruction. Involvement of the complement plays a certain but limited role in w-AIHA, whereas c-AIHA is entirely complement-dependent. In this connection, therapies for different kinds of AIHA also differ. Thus, in w-AIHA the first-line drugs are glucocorticosteroids, while in c-AIHA their efficacy is disputable. In relapses and refractory haemolyses, administration of rituximab and splenectomy are recommended as the second-line therapy for w-AIHA. In c-AIHA, early administration of rituximab is recommended as the first-line therapy. The use of other immunosuppressive drugs (azathioprine, cyclophosphamide, cyclospirin A, mofetil mycophenolate) as alternative therapy is possible but their effectiveness is significantly lower.

Individual selection of dose and calculation of ratio for prevention of the introduction of the concentrate of coagulation factor VIII 23 patients with severe hemophilia A carried out the pharmacokinetic study. The half-life of exogenous factor VIII is 12 hours, registered in 12 (52%) patients, 7 boys (30%) - the half-life of factor VIII was 18 hours, in 4 (18%) children - 11 hours. Individual pharmacokinetic calculations allowed to carry out secondary prevention of spontaneous bleeding with multiplicity systematic introduction of a concentrate of coagulation factor VIII in 72, 96, or 48 hours, respectively, without bleeding complications. The combined change in parameters such as higher clearance (Cl) to 5,3 ml х kg^-1 х hour^-1, volume of distribution (Vd) to 79.0 ml х kg^-1 with the decline in the normalized measure of the area under the curve (AUCnorm) to 14.7 ml^-1 х h х kg and the recovery index (Recovery in vivo) to 1.27 % / ME х kg^-1 and half-life (T/) to 11.0 hours, characterizes a type of inhibitory response to the introduction of the concentrate of coagulation factor VIII.

The objective. The aim of the study was to assess photobiomodulation (PBM) efficacy in management of OM in children during chemotherapy with HD-MTX. Patients and methods. Thirty three children and adolescents with acute lymphoblastic leukemia and non-Hodgkin's lymphoma participated in randomized prospective control study. Ninety three episodes ofHD-MTX chemotherapy were assessed. All patients were administered supportive therapy adopted in the Center. Sixteen children have received PBM of oral mucosa with semiconductor laser with wave length X = 670 ± 0,02 nm in prophylactic (5,16 J/om²) and therapeutic regimens (from 5,16 J/om2 to 21,24 J/om²) during 46 episodes of CT. Results. Oral mucositis was registered in 10 patients (59%) receiving PBM compared to 14 (88 %) patients without this method of prophylactics (p = 0,118), during 30% and 67% of episodes of HD-MTX chemotherapy, respectively (p < 0,001). Frequency of severe oral mucositis in patients receiving PBM was 8% compared to 17% in patients without this method of prophylactics (p = 0,355). The mean for pain assessment according to the Visual Analogue Scale was 0,8 points (95% CI 0-1,8) and 2,3 points (95% CI 1,2-3,4), respectively (p = 0,021), the mean for Lansky score was 91 points (94-99) and 63 points (54-71), respectively (p < 0,001), median of OM duration was 9 days [4-19] and 10 days [2-24], respectively (p = 0,721).Analysis long-term results of treatment of the main disease using general survival rate did not differentiate in groups (p = 0,16). PBM did not have side effects, was well tolerated and easily adopted in the treatment protocols of the Center. The use of PBM decreased the cost of accompanying therapy for the episode of CT by 39%. Conclusion. PBM is safe, effective and available method that can be recommended for management of OM in children and adolescent during HD-MTX chemotherapy.

Wiscott-Aldrich syndrome (WAS) is a rare X-liked recessive disease caused by mutation of the WASP gene. Disease is characterized by microthrombocytopenia, disorders of cell-mediated and humoral immunity, bleeding, eczema, frequent occurrence of infections, autoimmune diseases and malignancies. The article presents recent evidence about the role of WASP protein in the mechanisms of haemostasis and immune defence, discusses hypotheses for molecular mechanisms of WAS pathogenesis and interrelations between particular WASP gene mutations and clinical manifestations of disease. The review is illustrated by a clinical case of WAS with untypical clinical manifestations and absence of microthrombocytopenia illustrates.