From the Research
Chronic neutrophilic leukemia (CNL) should be diagnosed based on a neutrophil count above 25 x 10^9/L, less than 10% immature granulocytes in the blood, and the presence of the CSF3R T618I mutation in most cases, and treated with hydroxyurea as first-line therapy at doses of 500-2000 mg daily to control neutrophil counts, with ruxolitinib (a JAK inhibitor) at 5-25 mg twice daily considered for patients who fail hydroxyurea, particularly in those with the CSF3R mutation, as recommended by the most recent study 1.
Diagnosis of CNL
- Requires a neutrophil count above 25 x 10^9/L
- Less than 10% immature granulocytes in the blood
- Presence of the CSF3R T618I mutation in most cases
Treatment of CNL
- Hydroxyurea as first-line therapy at doses of 500-2000 mg daily to control neutrophil counts
- Ruxolitinib (a JAK inhibitor) at 5-25 mg twice daily for patients who fail hydroxyurea, particularly in those with the CSF3R mutation
- Allogeneic hematopoietic stem cell transplantation offers the only potential cure for eligible patients with aggressive disease
Prognosis and Monitoring
- CNL has a poor prognosis with median survival of 2-3 years
- Progression to acute myeloid leukemia is a common complication
- Regular blood count monitoring is essential
- Patients should be alert to symptoms of disease progression such as increasing fatigue, night sweats, weight loss, or bone pain The disease's pathophysiology involves dysregulated neutrophil production due to mutations affecting the CSF3R signaling pathway, which explains why targeted therapies like JAK inhibitors show promise, as discussed in the study 1. Other studies, such as 2, 3, 4, and 5, provide additional information on the diagnosis and treatment of CNL, but the most recent and highest quality study 1 provides the best guidance for clinical practice.