This protocol addresses symptomatic classical hairy-cell leukaemia in patients who received initial purine analogue therapy — cladribine (2-CldA) or pentostatin (DCF) — but did not reach the expected complete response.
Patients present with symptomatic classical HCL characterised by bulky or progressive 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.
First-line therapy: purine analogue — cladribine (2-CldA) or pentostatin (DCF).
Escalation to this protocol is triggered when the expected endpoint — complete response (morphological absence of hairy cells in peripheral blood and bone marrow, normalisation of organomegaly, and normalisation of peripheral blood counts) — was not achieved.
The protocol involves a second course of 2-CldA, which may incorporate an additional agent, aimed at achieving a complete response. The full protocol — combination details, eligibility criteria, and sequencing — is available via the structured regimen below.
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].
In patients demonstrating a PR after the first course of 2-CldA, a second course should be repeated to achieve a CR at least 6 months after the end of the first course, with or without rituximab [IV, B] [26].
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