Approach to Hairy-Cell Leukemia in the New Therapeutic Era with Special Emphasis on Age and Comorbidities.

Background: Hairy-cell leukemia (HCL) is a rare chronic hematologic malignancy, generally presenting with pancytopenia, relative lymphocytosis, monocytopenia, and splenomegaly. Diagnosis is based on typical bone marrow evaluation with the BRAF-V600E mutation being present in almost 100% of cases of classical HCL.

Summary: Treatment usually involves the use of purine analogs (PAs) as first-line therapy. Novel targeting therapies have recently been included in the treatment of therapy-naive HCL such as PA combination with anti-CD20, BRAF inhibitors alone, or combined with anti-CD20. In relapse/refractory disease, other novel agents were studied as BRAF and MEK inhibitors, Bruton tyrosine kinase, or BCL2 inhibitors: all showed encouraging results. Most clinical trials and guidelines do not specify what the optimal approach is for patients with HCL in special situations as elderly population above 80 years old, very young patients below 40 years old, pregnant women, and when leukemia is presented with other comorbidities as active infection or vasculitis.

Key messages: In this current manuscript, we summarized our approach to HCL in the era of novel agents with special emphasis on age and comorbidities.