A Rare Case of Primary Myelofibrosis in a 14-Year-Old Patient With Congenital Asplenia
- Resource Type
- Authors
- Maliha Khan; Srdan Verstovsek; Amber M Yates; Cheng C. Yin; Kate J. Newberry
- Source
- Journal of Pediatric Hematology/Oncology. 39:e196-e198
- Subject
- Pediatrics
medicine.medical_specialty
Adolescent
Primary Immunodeficiency Diseases
media_common.quotation_subject
Computed tomography
03 medical and health sciences
0302 clinical medicine
Rare case
Humans
Medicine
Girl
Myelofibrosis
media_common
medicine.diagnostic_test
business.industry
Immunologic Deficiency Syndromes
Hematology
medicine.disease
Oncology
Primary Myelofibrosis
030220 oncology & carcinogenesis
Pediatrics, Perinatology and Child Health
Female
Congenital asplenia
Tomography, X-Ray Computed
business
Spleen
030215 immunology
- Language
- ISSN
- 1077-4114
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.