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DOI 10.1111/bjh.19831

Медицинская библиотека · British journal of haematology · 2024

Haematopoietic cell transplantation for 106 infants and preschoolers with acquired and inherited bone marrow failures.

Loth Gisele, Dumke Cilmara C K, Muratori Rafaella R, Pelegrina Polliany D, Peixoto Carolina M A, Bach Juliana L M, Nichele Samantha, Trennepohl Joanna P, Koliski Adriana, Mousquer Rebeca T G, Rodrigues Adriana M, Marchesini Rafael, Feitosa Margareth K, Pilonetto Daniela V, Gouvea Lara, Benini Fernanda M L, Pirolli Emanuele S, Tutumi Rebeca A F, Lima Alberto C M, Pasquini Ricardo, Bonfim Carmem

СтатьяМногоцентровое исследованиеПеревод в очереди

Аннотация

Aplastic anaemia in infants and young children presents unique challenges due to high prevalence of inherited bone marrow failure syndromes (IBMFS) in this age group. The objective of this study is assessing clinical characteristics and outcomes of haematopoietic cell transplantation in children ≤5 years with bone marrow failure syndromes. We analysied 106 patients (66% males), median age 4.6 years, including 40 with Fanconi anaemia (FA), 32 with Acquired Severe Aplastic anaemia (aSAA), 15 with Diamond-Blackfan Anaemia, 11 with Amegakaryocytic Purpura and 8 with other IBMFS. Molecular testing was limited (39%), with 25.4% confirmed genetically. Retrospective longitudinal study across three paediatric transplantation centres (1982-2020). Overall survival (OS) was 76.4% over a median 10-year follow-up. OS rates were similar between aSAA and IBMFS (FA 77.5%, other IBMFS 76.5%). Transplant-related mortality (TRM) was lower in aSAA (9.4%) compared with IBMFS (16.2%). Recent years showed improved outcomes, with TRM declining post-2010. Choice of stem cell source impacted OS, favouring bone marrow over umbilical cord, but showing encouraging results with haploidentical. Late complications were common, including endocrine-metabolic issues and delayed neuropsychomotor development. Diagnosing and managing bone marrow failures in young children pose significant challenges. Despite advancements in transplant practices, ongoing vigilance and comprehensive care are necessary to improve long-term survival rates.

Для цитирования: Loth Gisele, Dumke Cilmara C K, Muratori Rafaella R, Pelegrina Polliany D, Peixoto Carolina M A, Bach Juliana L M, Nichele Samantha, Trennepohl Joanna P, Koliski Adriana, Mousquer Rebeca T G, Rodrigues Adriana M, Marchesini Rafael, Feitosa Margareth K, Pilonetto Daniela V, Gouvea Lara, Benini Fernanda M L, Pirolli Emanuele S, Tutumi Rebeca A F, Lima Alberto C M, Pasquini Ricardo, Bonfim Carmem. Haematopoietic cell transplantation for 106 infants and preschoolers with acquired and inherited bone marrow failures.. British journal of haematology. 2024. DOI: 10.1111/bjh.19831.

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