Hairy-cell leukemia
ICD-10 C91.4 · ICD-11 2A82.2

Treatment of Hairy-Cell Leukemia with Symptomatic Splenomegaly and Cytopaenias

Classical hairy cell leukaemia requires active treatment when the disease becomes symptomatic. This protocol defines the approach for patients with specific measurable disease findings — including enlarged spleen causing symptoms and blood count abnormalities.

Clinical scenario

This protocol applies to patients with classical hairy cell leukaemia presenting with symptomatic disease: bulky or progressive symptomatic splenomegaly; cytopaenias (haemoglobin <10 g/dl and/or platelets <100 × 109/l and/or neutrophils <1 × 109/l); recurrent or severe infections; and/or systemic symptoms. The patient must not be pregnant.

Treatment approach (overview only)

For eligible patients in this clinical situation, the treatment pathway involves a transplant-based intervention — the full eligibility criteria, specific approach, and treatment sequence are available in the complete protocol.

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References

DOI: 10.1093/annonc/mdv200

Treatment should be initiated in patients with symptomatic disease manifested by bulky or progressive, symptomatic splenomegaly cytopaenias (haemoglobin <10 g/dl and/or platelets <100 × 109/l and/or neutrophils <1 × 109/l), recurrent or severe infections and/or systemic symptoms [II, A] [17, 18].

Allogeneic stem cell transplantation has a potential role in younger, heavily pretreated HCL patients who have had multiple relapses and are refractory to purine analogues and rituximab [V, C] [50, 51].

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