title: A Rare Case of Primary Myelofibrosis in a 14-Year-Old Patient With Congenital Asplenia.
abstract: Primary myelofibrosis (PMF) is rarely diagnosed in children, and in most cases in children younger than 3 years old. Pediatric PMF generally follows a benign course and is usually managed supportively with blood transfusions and prophylactic antibiotics for infections. We present a case of a 17-year-old girl diagnosed with PMF at the age of 14 years. A computed tomography scan performed at the time of an appendectomy showed congenital asplenism. To our knowledge, this is only the third case of myelofibrosis and congenital asplenism to be reported in the literature. Whether asplenism contributed to the development of myelofibrosis is not known.
authors.affiliation: Departments of *Leukemia †Hematopathology, The University of Texas MD Anderson Cancer Center ‡Department of Pediatrics, Section of Hematology-Oncology, Texas Children's Hospital, Houston, TX.
authors.forename: Maliha
authors.initials: M
authors.lastname: Khan
authors.forename: Cheng C
authors.initials: CC
authors.lastname: Yin
authors.forename: Amber
authors.initials: A
authors.lastname: Yates
authors.forename: Kate J
authors.initials: KJ
authors.lastname: Newberry
authors.forename: Srdan
authors.initials: S
authors.lastname: Verstovsek
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging*; D000081207:Primary Immunodeficiency Diseases; D055728:Primary Myelofibrosis / Q000150:complications / Q000175:diagnosis*; D013154:Spleen / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed