Abstract
Introduction: Primary immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children, with a reported incidence of 1.1-12.5 cases per 100 000 children. However, currently, there are several definitions of ITP, as well as diagnostic and therapeutic approaches. Objective: To develop an evidence-based clinical practice guideline (CPG) to standardize the definition of ITP and, in this way, reduce the variability of its diagnosis, and to provide indications for the treatment of acute, persistent, and chronic ITP in patients under 18 years of age. Materials and methods: The CPG was prepared by a multidisciplinary group that followed the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) guidelines for developing CPGs, formulated PICO clinical questions, and conducted systematic reviews. GRADE evidence profiles were created and recommendations, with their corresponding level of evidence and strength, were made after a panel of experts assessed the benefit-risk balance, the quality of evidence, the patients’ values and preferences, and the context in which they should be implemented. Results: A total of 23 recommendations were made to pediatricians, hematologists, and health professionals working in emergency services for treating acute, persistent, and chronic ITP. Overall, the CPG has low quality of evidence, and the recommendations were made in order to improve the success rate of ITP treatment and the prognosis of children with this condition. Conclusions: Although ITP is the main cause of thrombocytopenia in pediatrics, to date there is not enough high-quality evidence that supports the recommendations presented here for its proper classification and treatment in children. Thus, further studies providing high-quality evidence on this issue are required.
Highlights
Primary immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children, with a reported incidence of 1.1-12.5 cases per 100 000 children
A total of 23 recommendations were made to pediatricians, hematologists, and health professionals working in emergency services for treating acute, persistent, and chronic ITP
ITP is the main cause of thrombocytopenia in pediatrics, to date there is not enough high-quality evidence that supports the recommendations presented here for its proper classification and treatment in children
Summary
Primary immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children, with a reported incidence of 1.1-12.5 cases per 100 000 children. Conclusions: ITP is the main cause of thrombocytopenia in pediatrics, to date there is not enough high-quality evidence that supports the recommendations presented here for its proper classification and treatment in children. Primary immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in pediatric population It is an immune disorder characterized by isolated, temporary or persistent thrombocytopenia (platelet count below 100x109/L) without any other underlying cause or disease.[1,2,3] The incidence reported is between 1.1 to 12.5 cases per 100 000 children and is more frequently observed between 2 and 8 years of age.[4] Clinical manifestations in this population are generally mild, but may be severe in approximately 3% of the cases, ranging from no symptoms or minimal cutaneous manifestations to life-threatening bleeding.[3,5]. For the purposes of this paper, the name “Primary Immune Thrombocytopenia” (ITP) will be used.[5,7]
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