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Author Notes:

Correspondence to: Oregon Health and Sciences University, Knight Cancer Institute, 3181 SW Sam Jackson Park Road, UHN73C, Portland, OR 97239, USA. Tel.: +1 503 494 7894; fax: +1 503 494 3688. 5Tdaok@ohsu.edu5T


Research Funding:

KTD receives support from the OHSU Knight Cancer Institute and the National Institutes of Health, National Heart, Lung and Blood Institute (1K08HL111280).

BJD is supported by the Howard Hughes Medical Institute. JWT is supported by Grants from the V Foundation for Cancer Research, the Leukemia and Lymphoma Society, the Gabrielle׳s Angel Foundation for Cancer Research, and the National Cancer Institute (5R00CA151457 and 1R01CA183974).


  • Atypical chronic myeloid leukemia
  • Chronic neutrophilic leukemia
  • Colony-stimulating factor 3 receptor
  • Ruxolitinib

Significant clinical response to JAK1/2 inhibition in a patient with CSF3R-T618I-positive atypical chronic myeloid leukemia


Journal Title:

Leukemia Research Reports


Volume 3, Number 2


, Pages 67-69

Type of Work:

Article | Final Publisher PDF


Mutations in CSF3R (colony-stimulating factor 3 receptor) are frequent oncogenic drivers in chronic neutrophilic leukemia (CNL) and atypical chronic myeloid leukemia (aCML). Here we describe a 75 year old man who was diagnosed with CSF3R-T618I-positive atypical CML. He presented with leukocytosis, anemia, and thrombocytopenia and developed massive splenomegaly and severe constitutional symptoms. Hydroxyurea was given over a 6 month period but failed to provide any measureable clinical benefit. Eventually, he was treated with ruxolitinib, an FDA-approved JAK1/2 inhibitor, which resulted in dramatic improvement of his blood counts. He also had significant reduction of spleen volume and constitutional symptoms. This case highlights the need for a clinical trial to interrogate JAK1/2 as a potential molecular target in CNL and aCML in patients with or without CSF3R mutation. A clinical trial evaluating the safety and efficacy of ruxolitinib for this patient population is registered at ClinicalTrials.gov (NCT02092324).

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© 2014 The Authors

This is an Open Access work distributed under the terms of the Creative Commons Attribution-NonCommerical-NoDerivs 3.0 Unported License (http://creativecommons.org/licenses/by-nc-nd/3.0/).

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