title: Systemic mastocytosis in a child with t(8;21) acute myeloid leukemia.
abstract: Mastocytosis is primarily limited to the cutaneous variant in pediatric patients. Systemic mastocytosis (SM) has been associated with t(8;21) acute myeloid leukemia (AML) in adults. We provide the first report of a child with t(8;21) AML, diagnosed with asymptomatic SM following four cycles of chemotherapy. Unlike most adults with SM/AML, she was not found to have a c-KIT (D816V) mutation. SM persisted in the bone marrow after completion of chemotherapy, and her AML relapsed 9 months off-treatment. Although she achieved a second remission, mastocytosis persists in the marrow. Pediatric patients with t(8;21) AML/SM may represent a high-risk group despite favorable cytogenetics.
authors.affiliation: Division of Pediatric Hematology/Oncology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York 10467, USA.
authors.forename: Kris M
authors.initials: KM
authors.lastname: Mahadeo
authors.forename: Lucia
authors.initials: L
authors.lastname: Wolgast
authors.forename: Christine
authors.initials: C
authors.lastname: McMahon
authors.forename: Peter D
authors.initials: PD
authors.lastname: Cole
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D002469:Cell Separation; D002648:Child; D002869:Chromosome Aberrations; D002898:Chromosomes, Human, Pair 8; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D015470:Leukemia, Myeloid, Acute / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D034721:Mastocytosis, Systemic / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy