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

• Published in: Pediatric blood & cancer Vol. 70; no. 4; pp. e30144 - n/a
• Main Authors: Kim, Taylor Olmsted, Curry, Choladda V., Wiszniewska, Joanna, Elghetany, M. Tarek, Satter, Lisa R. Forbes, Grimes, Amanda B., Despotovic, Jenny M.
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01-04-2023
• Subjects: Adolescent; Adult; Autoimmune Diseases - diagnosis; Autoimmunity; Child; Fibrosis; Hematology; Humans; Leukopenia; Lupus Erythematosus, Systemic; Myelofibrosis; Oncology; Pediatrics; Primary Myelofibrosis - pathology; Systemic lupus erythematosus; Tertiary Care Centers
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.30144

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.