Classical hairy cell leukaemia (HCL) is managed expectantly until the disease becomes symptomatic. When specific haematological or clinical thresholds are crossed, treatment becomes indicated and a structured protocol applies.
Treatment is indicated in classical HCL when symptomatic disease is present, manifested by one or more of the following:
Patient is not pregnant.
For patients refractory to purine analogue therapy, the protocol includes anti-CD22 directed treatment options among others — the full regimen details all indications, sequencing, and eligibility criteria.
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].
Other promising drugs active in purine analogue refractory HCL patients include moxetumomab pasudotox, an anti-CD22 recombinant immunotoxin, and vemurafenib, a BRAF V600E inhibitor [44–46].
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