Treatment

Many patients require no specific treatment.

Treatment should be initiated in patients with symptomatic disease (e.g., symptomatic splenomegaly or hepatomegaly; constitutional symptoms [excessive fatigue; unexplained weight loss >10% within prior 6 months]) (1)

A purine analogue (cladribine or pentostatin) is standard first-line treatment for HCL (2)

If patients are unsuitable for initial treatment with a purine analogue (e.g., due to frailty, renal insufficiency, active infection), vemurafenib (a BRAF inhibitor) may be considered either alone or combined with an anti-CD20 monoclonal antibody (rituximab or obinutuzumab) (3)

Splenectomy may be effective in reversing pancytopenia. in some patients. If splenectomy is considered, patients should be immunised against Streptococcus pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis. (4)

Reference

1. Grever MR, Abdel-Wahab O, Andritsos LA, et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. Blood. 2017 Feb 2;129(5):553-60.
2. Parry-Jones N, Joshi A, Forconi F, et al. Guideline for diagnosis and management of hairy cell leukaemia (HCL) and hairy cell variant (HCL-V). Br J Haematol. 2020 Dec;191(5):730-7.
3. Park JH, Winer ES, Huntington​ SF, et al. First line chemo-free therapy with the BRAF inhibitor vemurafenib combined with obinutuzumab is effective in patients with HCL. Blood. 2021 Nov 23;138(suppl 1):43.
4. Centers for Disease Control and Prevention. Vaccine recommendations and guidelines of the ACIP​. Altered immunocompetence. August 2023 [internet publication].