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. 2023 Apr;70(4):e30144.
doi: 10.1002/pbc.30144. Epub 2023 Jan 20.

Pediatric autoimmune myelofibrosis: Experience from a large pediatric tertiary care center

Taylor Olmsted Kim  1   2 , Choladda V Curry  2   3 , Joanna Wiszniewska  4 , M Tarek Elghetany  2   3 , Lisa R Forbes Satter  2   5 , Amanda B Grimes  1   2 , Jenny M Despotovic  1   2
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Pediatric autoimmune myelofibrosis: Experience from a large pediatric tertiary care center

Taylor Olmsted Kim et al. Pediatr Blood Cancer. 2023 Apr.

Abstract

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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References

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