RESUMEN
INTRODUCTION: Although primary immune thrombocytopenia (ITP) is rare in childhood, it is the most frequent cause of thrombocytopenia. There have been attempts to establish risk factors to predict the progression of the disease in order to optimise its management, which has changed in recent years due to, among other reasons, specialised care. MATERIAL AND METHODS: A retrospective, observational and analytical study was conducted on patients diagnosed with ITP over a 3-year period in a Paediatric Haematology specialist clinic. RESULTS: From the epidemiological, clinical and analytical point of view, the characteristics of this group are similar to others. Most of the patients (23/31, 74.2%) had ITP for less than 12 months, with there being no serious complications related to the disease or the treatment received. It was established that risk factors were related to being slowly evolving (lower event-free survival (EFS)) with no statistical significance, female gender, age over 10 years, leukopenia absence of initial severe thrombocytopenia, and non-specialised care. The absence of a history of infection was significantly related to a lower EFS. CONCLUSIONS: The epidemiological and analytical risk factors for a slowly evolving ITP are the same that described in the literature. Patients treated before the beginning of specialised care also had a lower EFS. These data seem to support the current recommendation that rare diseases should be managed in specialised units.
Asunto(s)
Púrpura Trombocitopénica Idiopática/diagnóstico , Adolescente , Niño , Preescolar , Progresión de la Enfermedad , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Pronóstico , Púrpura Trombocitopénica Idiopática/complicaciones , Púrpura Trombocitopénica Idiopática/epidemiología , Púrpura Trombocitopénica Idiopática/terapia , Estudios Retrospectivos , Factores de Riesgo , España/epidemiologíaRESUMEN
INTRODUCCIÓN: La trombocitopenia inmune primaria (PTI) es poco frecuente en la infancia, pero es la causa más habitual de trombocitopenia. Se han intentado establecer factores de riesgo para predecir su evolución, con el objetivo de poder optimizar su manejo, que se ha modificado en los últimos años, debido, entre otros factores, a una atención más especializada. MATERIAL Y MÉTODOS: Estudio retrospectivo, observacional y analítico de los pacientes con PTI, en un periodo de 3 años, en una consulta especializada en Hematología Pediátrica. RESULTADOS: Desde el punto de vista epidemiológico, clínico y analítico, las características de esta serie son similares a las de otros grupos. La mayoría de los pacientes (23/31; 74,2%) presentaron una PTI de duración menor de 12 meses, sin complicaciones graves relacionadas con la enfermedad ni con el tratamiento. Se establecieron como factores de riesgo relacionados con una evolución tórpida (supervivencia libre de eventos [SLE] menor), sin alcanzar la significación estadística, el sexo femenino, la edad mayor de 10 años, la leucopenia, la ausencia de trombocitopenia grave inicial y la atención no especializada. La ausencia de antecedente de infección se relacionó significativamente con una SLE menor. CONCLUSIONES: Los factores de riesgo de evolución tórpida de PTI epidemiológicos y analíticos de este estudio coinciden con los descritos en la literatura. Presentaron una SLE menor los pacientes tratados antes del inicio de la atención especializada. Estos datos parecen apoyar la recomendación actual de que las enfermedades poco frecuentes, como esta, se controlen en unidades especializadas
INTRODUCTION: Although primary immune thrombocytopenia (ITP) is rare in childhood, it is the most frequent cause of thrombocytopenia. There have been attempts to establish risk factors to predict the progression of the disease in order to optimise its management, which has changed in recent years due to, among other reasons, specialised care. MATERIAL AND METHODS: A retrospective, observational and analytical study was conducted on patients diagnosed with ITP over a 3-year period in a Paediatric Haematology specialist clinic. RESULTS: From the epidemiological, clinical and analytical point of view, the characteristics of this group are similar to others. Most of the patients (23/31, 74.2%) had ITP for less than 12 months, with there being no serious complications related to the disease or the treatment received. It was established that risk factors were related to being slowly evolving (lower event-free survival (EFS)) with no statistical significance, female gender, age over 10 years, leukopenia absence of initial severe thrombocytopenia, and non-specialised care. The absence of a history of infection was significantly related to a lower EFS. CONCLUSIONS: The epidemiological and analytical risk factors for a slowly evolving ITP are the same that described in the literature. Patients treated before the beginning of specialised care also had a lower EFS. These data seem to support the current recommendation that rare diseases should be managed in specialised units