医学
骨髓纤维化
自身免疫
髓外造血
纤维化
儿科
骨髓
免疫学
病理
抗体
造血
干细胞
遗传学
生物
作者
Taylor Olmsted Kim,Choladda V. Curry,Joanna Wiszniewska,M. Tarek Elghetany,Lisa Forbes Satter,Amanda B. Grimes,Jenny M. Despotovic
摘要
Autoimmune myelofibrosis (AIMF) is a rare disorder characterized by cytopenias and autoimmunity, with characteristic bone marrow findings that include lymphocytic infiltration and fibrosis. AIMF is described predominantly in adult populations who have systemic lupus erythematosis (SLE), with scant pediatric cases described mainly in older adolescents with SLE. Here, we described the largest single-center pediatric experience of pediatric autoimmune myelofibrosis (PAIMF) series, demonstrating both similarities and distinctions from the adult experience. Patients overall respond well to steroid therapy, but these patients were significantly younger, infrequently carried a diagnosis of SLE, and causative genetic lesions were identified in many cases.
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