Classical Hairy Cell Leukaemia with Symptomatic Disease After Second-Line Treatment Failure

Clinical Scenario

This protocol addresses classical hairy cell leukaemia (HCL) presenting with active symptomatic disease, defined by one or more of the following features:

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 is not pregnant.

Previous Treatment — Failure Condition

The prior treatment line comprised a second course of 2-CldA, with or without rituximab, administered with the intent of achieving a complete response.

This protocol applies when that complete response was not achieved — representing failure of the preceding line and the need for a subsequent treatment strategy.

Treatment Approach (Partial Overview)

For patients in this situation, retreatment strategies involving nucleoside analogues and/or monoclonal antibody-based combinations may be considered, with agent selection depending on relapse characteristics. Additional options exist for selected patients who relapse after prior purine analogue therapy.

The complete evidence-based regimen — including full agent selection criteria, sequencing, and all available options — is available in the structured protocol below.

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].

Relapsed patients can be successfully retreated with 2-CldA or DCF if relapse occurs after 12–18 months [IV, B] [32].

The alternative nucleoside analogue can be used in early relapse within 2 years after the first-line treatment [31].

Rituximab at a dose of 375 mg/m² for 4–8 doses given weekly as i.v. infusions can be used in early relapsed patients [III, B] [34–36].

Outcomes for patients with recurrent HCL appear to be better when a combination of rituximab and 2-CldA or DCF is used rather than the purine analogue alone [III, B] [37, 38].

IFN-α is also a possible option for selected patients relapsing after purine analogue therapy [IV, B] [40, 41].

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