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1.
An. pediatr. (2003. Ed. impr.) ; 91(2): 127.e1-127.e10, ago. 2019. tab, graf
Artigo em Espanhol | IBECS-Express | ID: ibc-ET2-3942

RESUMO

La trombocitopenia inmune primaria, anteriormente conocida como púrpura trombocitopénica inmune, es una enfermedad cuyo manejo diagnóstico y terapéutico ha sido siempre controvertido. La Sociedad Española de Hematología y Oncología Pediátricas, a través del grupo de trabajo de la PTI, ha actualizado el documento con las recomendaciones protocolizadas para el diagnóstico y tratamiento de esta enfermedad, basándose en las guías clínicas disponibles actualmente, revisiones bibliográficas, ensayos clínicos y el consenso de sus miembros. El objetivo principal es disminuir la variabilidad clínica en los procedimientos diagnósticos y terapéuticos con el fin de obtener los mejores resultados clínicos, los mínimos efectos adversos y preservar la calidad de vida


Primary immune thrombocytopenia, formerly known as immune thrombocytopenic purpura, is a disease for which the clinical and therapeutic management has always been controversial. The ITP working group of the Spanish Society of Paediatric Haematology and Oncology has updated its guidelines for diagnosis and treatment of primary immune thrombocytopenia in children, based on current guidelines, bibliographic review, clinical assays, and member consensus. The main objective is to reduce clinical variability in diagnostic and therapeutic procedures, in order to obtain best clinical results with minimal adverse events and good quality of life

2.
An Pediatr (Barc) ; 91(2): 127.e1-127.e10, 2019 Aug.
Artigo em Espanhol | MEDLINE | ID: mdl-31178291

RESUMO

Primary immune thrombocytopenia, formerly known as immune thrombocytopenic purpura, is a disease for which the clinical and therapeutic management has always been controversial. The ITP working group of the Spanish Society of Paediatric Haematology and Oncology has updated its guidelines for diagnosis and treatment of primary immune thrombocytopenia in children, based on current guidelines, bibliographic review, clinical assays, and member consensus. The main objective is to reduce clinical variability in diagnostic and therapeutic procedures, in order to obtain best clinical results with minimal adverse events and good quality of life.


Assuntos
Púrpura Trombocitopênica Idiopática/terapia , Qualidade de Vida , Criança , Humanos , Púrpura Trombocitopênica Idiopática/diagnóstico
4.
An. pediatr. (2003. Ed. impr.) ; 89(3): 189.e1-189.e8, sept. 2018. graf, tab
Artigo em Espanhol | IBECS | ID: ibc-177094

RESUMO

La trombocitopenia inmune (PTI) es una entidad relativamente frecuente en pediatría. Aunque su evolución suele ser favorable en la mayoría de los casos, el manejo de aquellos pacientes en los que la enfermedad persiste es muy controvertido. Este artículo pretende, a través de una revisión de la literatura más reciente, responder a aspectos relacionados con el tratamiento de la PTI persistente, crónica y refractaria durante la infancia, haciendo especial énfasis en el papel del rituximab, la esplenectomía y los análogos de la trombopoyetina (ar-TPO) en la infancia. La aparición de los ar-TPO (eltrombopag y romiplostim) amplía el arsenal terapéutico de la PTI pediátrica. Además, tras haber demostrado un perfil de seguridad adecuado en ensayos clínicos, retrasa la indicación de esplenectomía o el uso de tratamientos asociados a mayor riesgo de complicaciones, como rituximab. No obstante, se recomienda que su manejo sea supervisado por centros con experiencia de cara a monitorizar complicaciones potenciales a medio y largo plazo ya descritas en el paciente adulto


Immune thrombocytopenia (ITP) is a relatively common disorder in childhood. Although it usually achieves spontaneous remission at this age, the management of persistent or chronic ITP in children is still controversial. The aim of this article is to address current controversies related to the treatment of persistent, chronic, and refractory ITP in children, including the role of rituximab and splenectomy, as well as focusing on a new approach with thrombopoietin receptor agonists (TPO-RAs). Eltrombopag and romiplostim are safe and useful drugs for paediatric ITP. These two TPO-RAs might delay surgery and other treatments such as rituximab. However, the potential side effects described in adult patients should be considered. Paediatric patients with refractory ITP, undergoing new treatments, should be supervised in specialised centres


Assuntos
Humanos , Lactente , Trombocitopenia/imunologia , Trombopoetina/administração & dosagem , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Rituximab/administração & dosagem , Fatores de Risco , Receptores de Trombopoetina/agonistas
5.
An Pediatr (Barc) ; 89(3): 189.e1-189.e8, 2018 Sep.
Artigo em Espanhol | MEDLINE | ID: mdl-30033108

RESUMO

Immune thrombocytopenia (ITP) is a relatively common disorder in childhood. Although it usually achieves spontaneous remission at this age, the management of persistent or chronic ITP in children is still controversial. The aim of this article is to address current controversies related to the treatment of persistent, chronic, and refractory ITP in children, including the role of rituximab and splenectomy, as well as focusing on a new approach with thrombopoietin receptor agonists (TPO-RAs). Eltrombopag and romiplostim are safe and useful drugs for paediatric ITP. These two TPO-RAs might delay surgery and other treatments such as rituximab. However, the potential side effects described in adult patients should be considered. Paediatric patients with refractory ITP, undergoing new treatments, should be supervised in specialised centres.


Assuntos
Púrpura Trombocitopênica Idiopática/terapia , Criança , Doença Crônica , Árvores de Decisões , Humanos , Púrpura Trombocitopênica Idiopática/diagnóstico
8.
J Pediatr Hematol Oncol ; 33(1): e43-5, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20829714

RESUMO

Children with Noonan syndrome (NS) are at increased risk of developing juvenile myelomonocytic leukemia (JMML) or a myeloproliferative disorder associated with NS (MPD/NS) resembling JMML in the first weeks of life; whereas JMML is an aggressive disorder requiring hematopoietic stem cell transplantation, MPD/NS may resolve without treatment and cases with spontaneous remission have also been reported. Two cases of NS with hematologic disorders are described. Diagnosis of the syndrome was confirmed by the identification of earlier reported germline missense mutations in the PTPN11 gene. Splenomegaly in 1 patient and leukocytosis, monocytosis and "in vitro" culture assays consistent with JMML in both were the most salient hematologic features. After a 24-month follow-up, these 2 infants continue to improve and JMML has been ruled out. Splenomegaly persists in 1 patient and monocytosis in both, but without signs of malignancy, thereby suggesting abnormal hematopoiesis or MPD/NS, as described in NS.


Assuntos
Transtornos Mieloproliferativos/complicações , Síndrome de Noonan/complicações , Seguimentos , Humanos , Lactente , Recém-Nascido , Leucemia Mielomonocítica Juvenil/genética , Leucemia Mielomonocítica Juvenil/terapia , Masculino , Mutação de Sentido Incorreto , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Transtornos Mieloproliferativos/terapia , Síndrome de Noonan/diagnóstico , Síndrome de Noonan/genética , Proteína Tirosina Fosfatase não Receptora Tipo 11/genética
9.
Haematologica ; 95(6): 936-41, 2010 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-20179088

RESUMO

BACKGROUND: The presence of minimal residual disease detected by polymerase chain reaction techniques prior to allogeneic hematopoietic stem cell transplantation has proven to be an independent prognostic factor for poor outcome in children with acute lymphoblastic leukemia. DESIGN AND METHODS: The aim of this study was to ascertain whether the presence of minimal residual disease detected by multiparametric flow cytometry prior to allogeneic hematopoietic stem cell transplantation is related to outcome in children acute lymphoblastic leukemia. Minimal residual disease was quantified by multiparametric flow cytometry at a median of 10 days prior to hematopoietic stem cell transplantation in 31 children (age range, 10 months to 16 years) with acute lymphoblastic leukemia. Thirteen patients were transplanted in first remission. Stem cell donors were HLA-identical siblings in 8 cases and matched unrelated donors in 23. Twenty-six children received a total body irradiation-containing conditioning regimen. According to the level of minimal residual disease, patients were divided into two groups: minimal residual disease-positive (>or=0.01%) (n=10) and minimal residual disease-negative (<0.01%) (n=21). RESULTS: Estimated event-free survival rates at 2 years for the minimal residual disease-negative and -positive subgroups were 74% and 20%, respectively (P=0.004) and overall survival rates were 80% and 20%, respectively (P=0.005). Bivariate analysis identified pre-transplant minimal residual disease as the only significant factor for relapse and also for death (P<0.01). CONCLUSIONS: The presence of minimal residual disease measured by multiparametric flow cytometry identified a group of patients with a 9.5-fold higher risk of relapse and a 3.2-fold higher risk of death than those without minimal residual disease. This study supports the strong relationship between pre-transplantation minimal residual disease measured by multiparametric flow cytometry and outcome following allogeneic hematopoietic stem cell transplantation and concur with the results of previous studies using polymerase chain reaction techniques.


Assuntos
Citometria de Fluxo/métodos , Transplante de Células-Tronco Hematopoéticas/métodos , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Leucemia-Linfoma Linfoblástico de Células Precursoras/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Neoplasia Residual , Leucemia-Linfoma Linfoblástico de Células Precursoras/radioterapia , Fatores de Risco , Taxa de Sobrevida/tendências , Transplante Homólogo , Resultado do Tratamento , Irradiação Corporal Total/tendências
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