Recent Progress in Chronic Neutrophilic Leukemia and Atypical Chronic Myeloid Leukemia

2017 ◽  
Vol 12 (5) ◽  
pp. 432-441 ◽  
Author(s):  
Kim-Hien T. Dao ◽  
Jeffrey W. Tyner ◽  
Jason Gotlib
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Recent Progress ◽  
Chronic Neutrophilic Leukemia ◽  
Atypical Chronic Myeloid Leukemia

scholarly journals Efficacy of Ruxolitinib in Patients With Chronic Neutrophilic Leukemia and Atypical Chronic Myeloid Leukemia

2020 ◽  
Vol 38 (10) ◽  
pp. 1006-1018 ◽  
Author(s):  
Kim-Hien T. Dao ◽  
Jason Gotlib ◽  
Michael M.N. Deininger ◽  
Stephen T. Oh ◽  
Jorge E. Cortes ◽  
...  
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Adverse Events ◽  
Myeloid Leukemia ◽  
Response Rate ◽  
Symptom Assessment ◽  
Complete Response ◽  
Serious Adverse Events ◽  
Chronic Neutrophilic Leukemia ◽  
Atypical Chronic Myeloid Leukemia ◽  
Grade 3

PURPOSE Colony-stimulating factor-3 receptor ( CSF3R)-T618I is a recurrent activating mutation in chronic neutrophilic leukemia (CNL) and to a lesser extent in atypical chronic myeloid leukemia (aCML) resulting in constitutive JAK-STAT signaling. We sought to evaluate safety and efficacy of the JAK1/2 inhibitor ruxolitinib in patients with CNL and aCML, irrespective of CSF3R mutation status. METHODS We conducted a phase II study of ruxolitinib in 44 patients (21 CNL and 23 aCML). The primary end point was overall hematologic response rate (ORR) by the end of 6 continuous 28-day cycles for the first 25 patients enrolled. We considered a response as either partial (PR) or complete response (CR). We expanded accrual to 44 patients to increase our ability to evaluate secondary end points, including grade ≥ 3 adverse events, spleen volume, symptom assessment, genetic correlates of response, and 2-year survival. RESULTS ORR was 32% for the first 25 enrolled patients (8 PR [7 CNL and 1 aCML]). In the larger cohort of 44 patients, 35% had a response (11 PR [9 CNL and 2 aCML] and 4 CR [CNL]), and 50% had oncogenic CSF3R mutations. The mean absolute allele burden reduction of CSF3R-T618I after 6 cycles was greatest in the CR group, compared with the PR and no response groups. The most common cause of death is due to disease progression. Grade ≥ 3 anemia and thrombocytopenia were observed in 34% and 14% of patients, respectively. No serious adverse events attributed to ruxolitinib were observed. CONCLUSION Ruxolitinib was well tolerated and demonstrated an estimated response rate of 32%. Patients with a diagnosis of CNL and/or harboring CSF3R-T618I were most likely to respond.

Molecular characterization of atypical chronic myeloid leukemia and chronic neutrophilic leukemia

2015 ◽  
Vol 144 (11) ◽  
pp. 487-490
Author(s):  
Alicia Senín ◽  
Leonor Arenillas ◽  
Luz Martínez-Avilés ◽  
Concepción Fernández-Rodríguez ◽  
Beatriz Bellosillo ◽  
...  
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Molecular Characterization ◽  
Myeloid Leukemia ◽  
Chronic Neutrophilic Leukemia ◽  
Atypical Chronic Myeloid Leukemia

A Case of Atypical Chronic Myeloid Leukemia with the JAK2V617F Mutation

2011 ◽  
Vol 1 (4) ◽  
pp. 232
Author(s):  
Ju Yeon Kim ◽  
Se Ryeon Lee ◽  
Myung-Hyun Nam ◽  
Soo-Young Yoon ◽  
Chae Seung Lim ◽  
...  
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Jak2v617f Mutation ◽  
Atypical Chronic Myeloid Leukemia
Sign in / Sign up

Export Citation Format

Share Document