Prognosis and management

• Incurable but generally stable benign disease. Patients with NK phenotype (more common in Japan) or a CD3+ CD56+ clonal disorder established by TCR rearrangements, i.e. a true leukaemia, may have a more aggressive course. Patients with polyclonal disease associated with rheumatoid factor and modest neutropenia may run a more benign course.

• Care is essentially supportive with prompt treatment of infection with appropriate broad spectrum antibiotics.

• G-CSF may be of value in symptomatic chronic neutropenia. Corticosteroids in modest dosage may improve neutropenia but can predispose to infection, including fungal infections.

• Immunosuppression with low dose methotrexate (10mg/m2 PO weekly), cyclosporin (2mg/kg PO bd) or cyclophosphamide (100mg PO od) has been effective in ~50% of patients with persistent severe neutropenia.

• The rare patient with an aggressive course has a poor prognosis and lymphoma-type regimens show little benefit.

Blood film showing large granular lymphocytes in LGL leukaemia.

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