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

Author:

Kim Taylor Olmsted12,Curry Choladda V.23,Wiszniewska Joanna4,Elghetany M. Tarek23,Satter Lisa R. Forbes25,Grimes Amanda B.12,Despotovic Jenny M.12

Affiliation:

1. Texas Children's Cancer and Hematology Center Houston Texas USA

2. Departments of Pediatrics and Pathology & Immunology Baylor College of Medicine Houston Texas USA

3. Texas Children's Hospital Department of Pathology Houston Texas USA

4. Departments of Pathology and Molecular and Medical Genetics Oregon Health & Science University Portland Oregon USA

5. Immunology Allergy and Retrovirology and William T. Shearer Texas Children's Hospital Center for Human Immunobiology Houston Texas USA

Abstract

AbstractAutoimmune 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.

Publisher

Wiley

Subject

Oncology,Hematology,Pediatrics, Perinatology and Child Health

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