Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP)

再生障碍性贫血 医学 儿科 联想(心理学) 贫血 内科学 骨髓 心理学 心理治疗师
作者
Angela Guarina,Piero Farruggia,E Mariani,Paola Saracco,Angelica Barone,Daniela Onofrillo,Simone Cesaro,Rosa Angarano,Walter Barberi,Sonia Bonanomi,Paola Corti,Barbara Crescenzi,Gianluca Dell’Orso,Antonia De Matteo,G. Giagnuolo,Anna Paola Iori,Saverio Ladogana,A. Lucarelli,Michela Lupia,Baldassare Martire
出处
期刊:Blood Cells Molecules and Diseases [Elsevier BV]
卷期号:108: 102860-102860 被引量:3
标识
DOI:10.1016/j.bcmd.2024.102860
摘要

Acquired aplastic anemia (AA) is a rare heterogeneous disorder characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2–3 per million population per year in the Western world, but 3 times higher in East Asia. Survival in severe aplastic anemia (SAA) has improved significantly due to advances in hematopoietic stem cell transplantation (HSCT), immunosuppressive therapy, biologic agents, and supportive care. In SAA, HSCT from a matched sibling donor (MSD) is the first-line treatment. If a MSD is not available, options include immunosuppressive therapy (IST), matched unrelated donor, or haploidentical HSCT. The purpose of this guideline is to provide health care professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary evidence-based document prepared by a group of pediatric hematologists of the Bone Marrow Failure Study Group of the Italian Association of Pediatric Hemato-Oncology (AIEOP) was discussed, modified and approved during a series of consensus conferences that started online during COVID 19 and continued in the following years, according to procedures previously validated by the AIEOP Board of Directors.
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