Hairy cell leukaemia is a rare chronic B-cell lymphoproliferative disorder characterised by a long natural course with, in most of cases, an excellent response to a single course of purine analogue monochemotherapy. Making the right diagnosis, excluding the chemo resistant variant form of hairy cell leukaemia, and making progresses in the treatment of relapsing and/or refractory disease remains challenging up to date. In recent years, exciting results with new agents are emerging and clinical trials are ongoing to optimize the management of hairy cell leukaemia and its variant form.

(BELG J HEMATOL 2017;8(6):222–8)