Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up

Blood Cancer J. 2020 May 27;10(5):62. doi: 10.1038/s41408-020-0328-z.

Abstract

In total, 279 patients with hairy-cell leukemia (HCL) were analyzed, with a median follow-up of 10 years. Data were collected up to June 2018. We analyzed responses to treatment, relapses, survival, and the occurrence of second malignancies during follow-up. The median age was 59 years. In total, 208 patients (75%) were treated with purine analogs (PNAs), either cladribine (159) or pentosatin (49), as the first-line therapy. After a median follow-up of 127 months, the median overall survival was 27 years, and the median relapse-free survival (RFS) was 11 years. The cumulative 10-year relapse incidence was 39%. In patients receiving second-line therapy, the median RFS was 7 years. For the second-line therapy, using the same or another PNA was equivalent. We identified 68 second malignancies in 59 patients: 49 solid cancers and 19 hematological malignancies. The 10-year cumulative incidences of cancers, solid tumors, and hematological malignancies were 15%, 11%, and 5.0%, respectively, and the standardized incidence ratios were 2.22, 1.81, and 6.67, respectively. In multivariate analysis, PNA was not a risk factor for second malignancies. HCL patients have a good long-term prognosis. PNAs are the first-line treatment. HCL patients require long-term follow-up because of their relatively increased risk of second malignancies.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Agents / therapeutic use*
  • Cladribine / therapeutic use*
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Humans
  • Leukemia, Hairy Cell / drug therapy*
  • Leukemia, Hairy Cell / epidemiology
  • Male
  • Middle Aged
  • Pentostatin / therapeutic use*
  • Treatment Outcome

Substances

  • Antineoplastic Agents
  • Pentostatin
  • Cladribine