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

Treatment of Classical Hairy-Cell Leukemia with Symptomatic Splenomegaly and Cytopenias

In classical hairy-cell leukemia, treatment is indicated once the disease produces symptoms. This protocol applies to patients who meet specific criteria for symptomatic, active disease requiring intervention.

Clinical scenario Classical HCL with symptomatic disease manifested by bulky or progressive splenomegaly, cytopenias (haemoglobin <10 g/dl and/or platelets <100 × 109/l and/or neutrophils <1 × 109/l), recurrent or severe infections, and/or systemic symptoms. Patient is not pregnant.
Treatment approach This protocol outlines combination regimen options for eligible patients in this setting — including regimens applicable in relapsed or refractory disease. The complete list of agents, sequencing, and cycle structure is available in the full 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].

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].

Fludarabine at a dose of 40 mg/m2 oral (p.o.) for five consecutive days in combination with an i.v. injection of 375 mg/m2 rituximab on day 1, every 28 days for four cycles, can be a therapeutic option in relapsed or refractory patients previously treated with 2-CldA [IV, B].

Bendamustine at 70–90 mg/m2 combined with rituximab is another therapeutic option in multiply relapsed/refractory HCL, and could be considered in HCL patients after the failure of standard therapies [IV, B] [43].

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