TY - JOUR
T1 - Pediatric autoimmune myelofibrosis
T2 - Experience from a large pediatric tertiary care center
AU - Kim, Taylor Olmsted
AU - Curry, Choladda V.
AU - Wiszniewska, Joanna
AU - Elghetany, M. Tarek
AU - Satter, Lisa R.Forbes
AU - Grimes, Amanda B.
AU - Despotovic, Jenny M.
N1 - Funding Information:
Taylor Olmsted Kim receives consultancy fees from Novartis. Lisa R. Forbes Satter receives consultancy fees from Enzyvant, CSL Behring, Takeda, Grifols, ADMA, and Horizon. Jenny M. Despotovic receives consultancy fees, honoraria, and research support from Novartis, honoraria from Dova and Amgen, and royalties from Uptodate.
Publisher Copyright:
© 2023 Wiley Periodicals LLC.
PY - 2023
Y1 - 2023
N2 - 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.
AB - 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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U2 - 10.1002/pbc.30144
DO - 10.1002/pbc.30144
M3 - Article
C2 - 36661251
AN - SCOPUS:85147029269
SN - 1545-5009
JO - Medical and Pediatric Oncology
JF - Medical and Pediatric Oncology
ER -