Improved survival in hairy cell leukaemia over three decades: A SEER database analysis of prognostic factors

摘要

Hairy cell leukaemia (HCL) is a rare B cell malignancy characterized by a median age at diagnosis of 55 years, a Caucasian male preponderance, and a varivble clinical course (Grever, 2010). Initial therapeutic options were limited and outcomes poor with a median survival in the 1980s of 4-6 years (Grever & Lozanski, 2011). However, therapies have improved significantly over the last three decades with the use of alpha interferon (IFN-alpha) and the purine nucleoside analogues pentostatin and cladribine in 1984, 1986, and 1990 respectively (Quesada et al, 1984; Kraut et al, 1986; Piro et al, 1990; Else et al, 2009). The monoclonal antibody rituximab has shown promise in HCL, but is used primarily for relapsed or refractory patients, those who are not deemed candidates for purine analogs, or in an attempt to eradicate minimal residual disease (Cervetti et al, 2004; Ravandi, 2011; Ravandi et al, 2011). Although response rates and remission durations have improved, less is known about the impact of therapeutic advances on overall survival in the general population or demographic factors influencing outcomes. Such information is difficult to gain from clinical trials, which are influenced by patient selection, small patient numbers and short duration of follow up.