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 ?
Since more than 80% of white cells in blood and marrow were mature neutrophils, the diagnosis of chronic neutrophilic leukaemia was thought of.
Possibility of chronic neutrophilic leukaemia was thought of, but the presence of the BCR-ABL fusion gene confirmed the diagnosis of Chronic Myeloid leukaemia.
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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