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Blood, 15 November 2000, Vol. 96, No. 10, pp. 3647-3649

BRIEF REPORT

Spontaneous remission of granulocyte colony-stimulating factor-associated leukemia in a child with severe congenital neutropenia

Sima Jeha, Ka Wah Chan, Andrew G. Aprikyan, W. Keith Hoots, Steven Culbert, Hallie Zietz, David C. Dale, and Maher Albitar

From the Departments of Pediatrics and Hematopathology, University of Texas M.D. Anderson Cancer Center, Houston, TX, and the Department of Medicine, University of Washington, Seattle, WA.

Leukemia is observed with increased frequency in patients with severe congenital neutropenia (SCN). In the past decade, recombinant human granulocyte colony-stimulating factor (rh G-CSF) has prolonged the survival of patients with SCN increasingly reported to have leukemias. In this communication acute myelogenous leukemia (AML) associated with a mutation of the G-CSF receptor (G-CSF-R) developed in a patient with SCN maintained on long-term G-CSF therapy. The blast count in the blood and bone marrow fell to undetectable levels twice on withholding G-CSF and without chemotherapy administration, but the mutant G-CSF-R was detectable during this period. The patient subsequently underwent successful allogeneic bone marrow transplantation. After transplantation, the patient's neutrophil elastase (ELA-2) mutation and G-CSF-R mutation became undetectable by polymerase chain reaction. This report provides novel insights on leukemia developing in congenital neutropenia.

© 2000 by The American Society of Hematology.
 

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