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Chronic Neutrophilic Leukemia

Rare myeloproliferative neoplasm with sustained mature neutrophil expansion driven by CSF3R mutation

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Chronic Neutrophilic Leukemia?

Chronic neutrophilic leukemia is a rare BCR-ABL1-negative myeloproliferative neoplasm characterized by persistent mature neutrophilia exceeding 25,000 cells per microliter, hepatosplenomegaly, and absence of dysplasia. The CSF3R T618I activating mutation is present in most patients and supports the diagnosis.

Patients usually present in the seventh or eighth decade with fatigue, splenomegaly, and laboratory neutrophilia confirmed on bone marrow biopsy. World Health Organization criteria require exclusion of reactive neutrophilia, chronic myeloid leukemia, and atypical chronic myeloid leukemia.

Treatment options include hydroxyurea for cytoreduction, ruxolitinib that targets CSF3R-driven JAK signaling and provides symptomatic improvement, and interferon alpha in selected cases. Allogeneic stem cell transplantation is the only curative therapy and should be considered in fit patients with high-risk disease.

Symptoms

Sustained peripheral blood neutrophilia
Splenomegaly with abdominal discomfort
Fatigue and constitutional symptoms
Bone pain and pruritus in some patients
Bleeding or thrombosis in advanced disease

Risk Factors

CSF3R T618I activating mutation
Older adult age at presentation
Coexisting plasma cell dyscrasia in some cases
Absence of BCR-ABL1 fusion
Family history of myeloid neoplasm

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When persistent neutrophilia is unexplained
  • When splenomegaly causes symptoms
  • When bleeding or thrombosis occurs
  • When previous treatment loses effect
  • When transplant evaluation is appropriate

Treatment Methods

01
Bone marrow biopsy and CSF3R molecular testing
02
Hydroxyurea for cytoreduction
03
Ruxolitinib for symptomatic CSF3R-driven disease
04
Interferon alpha in selected patients
05
Allogeneic stem cell transplantation in eligible cases
06
Supportive transfusion and infection prophylaxis
07
Multidisciplinary review with hematology and transplant team

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Hematoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.