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A minority of patients develop purine analogue\u2010refractory disease. Targeted therapies have improved outcomes for such patients. Recently, the <jats:italic><jats:styled-content style=\"fixed-case\">BRAF<\/jats:styled-content><\/jats:italic> V600E mutation was identified in most patients with classical <jats:styled-content style=\"fixed-case\">HCL<\/jats:styled-content>, resulting in constitutive mitogen\u2010activated protein kinase pathway activation; impressive responses are achieved in heavily pre\u2010treated patients with <jats:styled-content style=\"fixed-case\">BRAF<\/jats:styled-content> inhibition. The <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>22\u2010targeted immunoconjugate moxetumomab pasudotox and <jats:styled-content style=\"fixed-case\">BTK<\/jats:styled-content> inhibitor ibrutinib also achieve responses in relapsed and refractory patients. <jats:styled-content style=\"fixed-case\">HCL<\/jats:styled-content> variant and the <jats:italic><jats:styled-content style=\"fixed-case\">IGHV<\/jats:styled-content>4\u201034<\/jats:italic> molecular variant of <jats:styled-content style=\"fixed-case\">HCL<\/jats:styled-content> lack <jats:italic><jats:styled-content style=\"fixed-case\">BRAF<\/jats:styled-content><\/jats:italic> mutation and have inferior outcomes with standard purine analogue therapy. The addition of rituximab to purine analogues achieves very high rates of minimal residual disease\u2010negative complete remission and improves outcomes for patients with <jats:styled-content style=\"fixed-case\">HCL<\/jats:styled-content> variant. 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