chronic neutrophilic leukaemia

chronic neutrophilic leukaemia

A rare myeloproliferative disorder characterised by prominent neutrophilia in peripheral blood, myeloid hyperplasia in the bone marrow, hepatosplenomegaly and an absence of the Philadelphia chromosome or BCR/ABL fusion gene.

Clinical findings
Hepatosplenomegaly; less commonly: pruritus, gout, mucocutaneous bleeding.

DiffDx
CML, neutrophilic-CML, myelodysplastic syndrome.

Management
Hydroxyurea to control leukocytosis and reduce splenomegaly; chemotherapy.

Prognosis
Uncertain; patients die in blast transformation or with progressive leucocytosis.
References in periodicals archive ?
Mr Duncan was diagnosed with Chronic Neutrophilic Leukaemia 20 years ago, a rare disorder which has no cure - there are only about 130 cases reported in the world.

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