RESUMEN
Approximately 5% of chronic immune thrombocytopenic purpura (ITP) manifests itself as symptomatic, severe thrombocytopenia requiring splenectomy. The surgical procedure increases the risk of serious hemorrhage, especially in patients refractory to platelet transfusions. Recombinant factor VIIa (rFVIIa) has been found to enhance thrombin generation on activated platelets and may be a promising agent in preventing life-threatening bleedings. The administration of rFVIIa in two patients with severe refractory ITP, who underwent splenectomy, is presented. Combined therapy with agents of different mechanisms of action could be useful in cases with the highest probability of bleeding.
Asunto(s)
Factor VIIa/uso terapéutico , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Preescolar , Enfermedad Crónica , Factor VIIa/administración & dosificación , Humanos , Lactante , Masculino , Recuento de Plaquetas , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/cirugía , Proteínas Recombinantes/administración & dosificación , Proteínas Recombinantes/uso terapéutico , Índice de Severidad de la Enfermedad , Esplenectomía , Resultado del TratamientoRESUMEN
AIM OF THE STUDY: Measurement of c-myb expression in leukaemia cells in children and in normal cells of healthy controls. MATERIAL AND METHODS: 37 patients, 23 boys and 14 girls with acute leukaemia, aged 1-17 years, were included in the study (32 with acute lymphoblastic leukaemia and 5 with acute myeloblasts leukaemia) Control group consisted of 17 healthy children, 8 boys and 9 girls, 4-18 years old. After the isolation of mononuclear cells from bone marrow I peripheral blood mRNA was isolated, then with the use of reverse transcriptase cDNA was synthesized. The level of expression of c-myb was analyzed with polymerase chain reaction method. The final result was analyzed as the ratio between fluorescence of c-myb gene and the control gene. RESULTS: Mean level of expression of c-myb gene in leukaemia cells was statistically significantly higher than in the control group (0.71+/-0.53 vs. 0.51+/-0.22; p=0.05), as well as c-myb level between leukaemia cells in relapse cases and controls (0.83+/-0.23 vs. 0.51+/-0.22; p=0.01). There was no difference between c-myb expression in different diagnosis. The comparison of c-myb expression in good and poor response group was not statistically significant (p=0.33). CONCLUSIONS: Possible influence of increased expression of c-myb gene in the promotion of leukaemia was found. The role of c-myb expression as a prognostic factor in acute leukaemias of children was not confirmed.