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VH4\u201034\u2010positive HCL cases are associated with a poor prognosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Treatment<\/jats:title><jats:p>Patients should be treated only if HCL is symptomatic. Chemotherapy with risk adapted therapy purine analogs (PNAs) are indicated in first\u2010line HCL patients. The use of chemo\u2010immunotherapy combining PNAs and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed\/refractory disease is based on the use of BRAF inhibitors (BRAFi) plus rituximab or MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22 or Bruton Tyrosine Kinase inhibitors (BTKi). However, the optimal sequence of the different treatments remains to be determined. 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