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

Treatment of Hairy-cell Leukemia with Symptomatic Splenomegaly and Cytopenias

Classical hairy cell leukaemia (HCL) requires active treatment once symptomatic disease is established. This protocol applies to non-pregnant patients presenting with specific haematological and clinical indicators that signal the need to initiate therapy.

Clinical scenario
Bulky or progressive, symptomatic splenomegaly; and/or cytopenias — haemoglobin <10 g/dl, platelets <100 × 109/l, or neutrophils <1 × 109/l; and/or recurrent or severe infections; and/or systemic symptoms. Patient is not pregnant.
Treatment approach

First-line therapy centres on a purine analogue. The choice of specific agent, administration route, and schedule is guided by individual patient factors — the complete regimen, including all options and criteria, is available in the structured protocol.

Treatment goals

The target is complete response: morphological absence of hairy cells in peripheral blood and bone marrow, with normalisation of any organomegaly and peripheral blood counts. Response assessment is conducted at a defined interval after completion of therapy.

Instant Access to Structured Evidence-Based Regimens

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].

Purine analogues, cladribine (2-CldA) or pentostatin (DCF), are recommended as initial treatment of symptomatic HCL patients who are young and fit (Figure 1) [II, A].

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