Publications (2)0 Total impact
Article: [Results of immunosupressive therapy in children with severe aplastic anaemia. Report of the Polish Paediatric Haematology Group].[show abstract] [hide abstract]
ABSTRACT: Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When there is no donor available, combined immunosuppressive therapy is given. evaluation of results of immunosupressive therapy in children with severe aplastic anaemia. Material and methods: SAA was diagnosed in 105 children (42 girls, 73 boys), aged 2-18 years, in the eleven haematological centres in Poland, between 1993-2007. All patients received the Severe Aplastic Anaemia Working Party of the EBMT protocol which included: antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone. Granulocyto- or granulocytomacrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84 or 112 and 180 of the therapy. complete remission occurred in 53 patients (51.5%), partial remission in 27 (24.7%), no response was obtained in 25 children (23.8%) on day 180, of the therapy. Period of observation was from 12 months to 12.5 years. During this time relapse occurred in 10 patients (9.5%). We observed 22 deaths: 8 early, during the first 3 months of IS and 14 after the first 3 months of immunosuppresive therapy (IS). At present 70 children (66.6%) are in first remission with lasts from 12 months to 12.5 years. The survival at 12.5-years is 78.6%. During the 12.5 years of follow-up we had two cases with a late clonal complication (PNH and MDS). Transformation to acute nonlymphoblastic leukaemia was observed in two of our patients. 1. Immunosuppresive therapy (IS) in children with SAA, without bone marrow family donors, is more effective after introduction of combined IS (12.5 years survival in this study was 80% for children with very severe aplastic anaemia (v SAA). 2. In our studies among the children followed up after IS therapy, there were: 1 case of periodic nocturnal haemoglobinuria (PNH), 1 case of myelodysplastic syndrome (MDS) and 2 cases of myeloid leukaemia (probability of incidence was 3.8%).Medycyna wieku rozwojowego 02/2008; 12(4 Pt 2):1092-7.
Article: [Prognosis of clinical course of primary brain tumours in children in relationship to the duration and characteristic features of initial clinical symptoms].[show abstract] [hide abstract]
ABSTRACT: Primary tumours of the central nervous system belong to the most frequently occurring neoplastic diseases in paediatric patients. During the initial phase of disease development, the clinical symptoms of brain tumours might suggest disorders of other organs and their diagnosis is frequently delayed in relation to therapeutic possibilities. The aim of the study was to analyse the characteristic features and duration of preliminary symptoms arising due to brain tumours in paediatric patients treated in a single centre and to try to assess their prognostic significance for recurrence and death. We performed a retrospective assessment of the characteristic features of preliminary symptoms of brain tumours in 81 paediatric patients (45 female, 35 male) in the age range of 1.5 month - 17.2 years. Those characteristic features included the duration of symptoms until diagnosis (Pre-Diagnostic Symptoms Interval, PSI) and their correlation with the tumour type, its localization and size at the moment of diagnosis. The mean duration of symptoms in the studied group was 3 months. In nearly 45% of patients PSI was longer then 3 months and in 17,5% it was over 6 months. The predominant preliminary disease symptoms were the symptoms of increased intracranial pressure (n=56, 69,1%) The longer PSI correlated with the disease recurrence rate (p=0.024) and death rate (p=0.04). When PSI was longer then 6 months, all the tumours diagnosed were larger then 30 mnm, however no relationship was found between PSI duration and the tumor size (p=0.35). There was no correlation between the tumour size and the frequency of death (p=0.8), but in patients with tumours smaller then 30 mm in their greatest dimension, the recurrence of the neoplastic process was more frequent. Duration of preliminary symptoms may have an effect on the tumour recurrence and on the rate of death in paediatric patients with brain tumours. Early diagnosis plays an evident role in prognosis.Medycyna wieku rozwojowego 9(3 Pt 2):567-78.