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.