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.