RESUMEN
AIM: To study clinical and laboratory characteristics of hepatitides and evaluate efficacy of immunosuppressive therapy and transplantation of the bone marrow in hepatitis-associated aplastic anemia (HAAA). MATERIAL AND METHODS: A retrospective analysis of case histories of children with HAAA was made. For all the patients standard tests for detection of aquired aplastic anemia and hepatitis were conducted. Transplantation of hemopoietic stem cells (THSC) from HLA-identical donors was made in 4 patients, 25 patients were treated with combined immunosuppressive therapy (antithymocytic globulin--ATG plus cyclosporin A -CsA), one patients received monotherapy with CsA, two--prednisolone and a short course of CsA, one child was untreated. RESULTS: Of 260 children admitted to hospital from April 1989 to July 2005 for aquired aplastic anemia, 33 (12.7%) met diagnostic criteria of HAAA. Boys to girls ratio was 267. Hepatitides were severe: median of alaninaminotransferase concentration was 1215 IU/l, aspartataminotransferase--789 IU/l, bilirubin--152.5 mcmol/l. Median of the interval from hepatitis symptoms to documentation of pancytopenia was 66 days (0-204 days). All four patients after THSC are alive for 30-72 months. Probability of complete remission after the first course of ATG+CsA is 0.72 +/- 0.09, probability of survival 0.81 +/- 0.07, median of the interval to transfusion independence--50 days. CONCLUSION: HAAA prognosis is good only in administration of up-to-date therapy. After seronegative hepatitis it is necessary to control hemogram parameters and in the presence of minimal cytopenia patients should be directed to hematological hospital.
Asunto(s)
Anemia Aplásica/diagnóstico , Anemia Aplásica/terapia , Trasplante de Células Madre Hematopoyéticas , Hepatitis/complicaciones , Terapia de Inmunosupresión , Adolescente , Alanina Transaminasa/sangre , Anemia Aplásica/etiología , Aspartato Aminotransferasas/sangre , Bilirrubina/sangre , Niño , Preescolar , Femenino , Humanos , Inmunosupresores/uso terapéutico , Lactante , Masculino , Pronóstico , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
AIM: To examine the pattern of changes in the count of peripheral granulocytes in children with aplastic anemias (AA), receiving a combined immunosuppressive therapy with antithymocytic globulin (ATG) and cyclosporin A in combination with granulocytic colony-stimulating factor (G-CSF). MATERIALS AND METHODS: 31 children (17 boys and 14 girls) aged 2-15 years (median 9 years) with newly diagnosed severe and very severe acquired AA took a combined immunosuppressive therapy with ATG and cyclosporin A in combination with G-CSF in an initial dose of 10 micrograms/kg a day. RESULTS: A three-linear and response was recorded in 19 (61%) children, an isolated granulocytic response was in 26 (84%). The interval median before the recovery of granulocytes to 1.5 x 10(9)/l and 5 x 10(9)/l was 19 and 38 days, respectively. CONCLUSION: Use of G-CSF may increase the count of granulocytes in the vast majority of patients with AA, without dramatic influence on the frequency of a three-linear response. Intermittent use of G-CSF may maintain the count of granulocytes long at the safe level and reduce the cost of treatment.
Asunto(s)
Anemia Aplásica/sangre , Anemia Aplásica/tratamiento farmacológico , Suero Antilinfocítico/uso terapéutico , Ciclosporina/uso terapéutico , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Granulocitos , Inmunosupresores/uso terapéutico , Recuento de Leucocitos , Adolescente , Suero Antilinfocítico/administración & dosificación , Niño , Preescolar , Ciclosporina/administración & dosificación , Interpretación Estadística de Datos , Quimioterapia Combinada , Femenino , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Humanos , Inmunosupresores/administración & dosificación , Masculino , Programas Informáticos , Factores de TiempoRESUMEN
Cyclosporin A (CA) was used in the treatment of 2 patients with acquired aplastic anemia. Upon reaching hematological remission these children had recurrence consequent to dose lowering or discontinuation of CA. The remission occurred again when full-dose CA treatment was resumed. Slow 2-year decrease in CA dose led to uneventful end of CA treatment without deterioration of blood picture.