title: Terminal acute myelogenous leukemia in a patient with congenital agranulocytosis.
abstract: Congenital agranulocytosis terminating in acute myelogenous leukemia has been previously reported in only two cases of adolescent males. We describe the clinical and laboratory features of a 13-year-old male with congenital agranulocytosis, treated with G-CSF with initial good neutrophil response, who subsequently developed acute myeloid leukemia. This rare complication may define a preleukemic subset of patients for whom G-CSF therapy is ineffective. The diagnostic challenges of this case are presented.
authors.affiliation: Department of Pediatrics, University of California, Los Angeles.
authors.forename: W Y
authors.initials: WY
authors.lastname: Wong
authors.forename: D
authors.initials: D
authors.lastname: Williams
authors.forename: M L
authors.initials: ML
authors.lastname: Slovak
authors.forename: B
authors.initials: B
authors.lastname: Charak
authors.forename: A
authors.initials: A
authors.lastname: Mazumder
authors.forename: D
authors.initials: D
authors.lastname: Snyder
authors.forename: D R
authors.initials: DR
authors.lastname: Powars
authors.forename: R K
authors.initials: RK
authors.lastname: Brynes
mesh_terms: D000293:Adolescent; D000380:Agranulocytosis / Q000150:complications* / Q000151:congenital / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D015321:Gene Rearrangement; D006801:Humans; D017403:In Situ Hybridization; D007621:Karyotyping; D015470:Leukemia, Myeloid, Acute / Q000150:complications* / Q000401:mortality / Q000473:pathology; D008297:Male; D013234:Stem Cells / Q000502:physiology