Terminal acute myelogenous leukemia in a patient with congenital agranulocytosis.
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.
Wong
W Y
WY
Department of Pediatrics, University of California, Los Angeles.
Williams
D
D
Slovak
M L
ML
Charak
B
B
Mazumder
A
A
Snyder
D
D
Powars
D R
DR
Brynes
R K
RK
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