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Ibrutinib for the treatment of Bing-Neel syndrome: A multicenter study

Jorge J. Castillo, Gilad Itchaki, Jonas Paludo, Marzia Varettoni, Christian Buske, Toby A. Eyre, Julio C. Chavez, Kenneth H. Shain, Samar Issa, M. Lia Palomba, Oren Pasvolsky, David Simpson, Dipti Talaulikar, Constantine S. Tam, Alessandra Tedeschi, Stephen M. Ansell, Lakshmi Nayak and Steven P. Treon

Key points

  • Ibrutinib was administered to 28 patients with Bing-Neel syndrome (BNS).

  • Ibrutinib showed rapid and durable symptomatic and radiological responses in patients with BNS.

Abstract

The treatment of patients with Bing-Neel syndrome (BNS) is not standardized. We included patients with Waldenstrom macroglobulinemia (WM) and a radiological and/or cytological diagnosis of BNS treated with ibrutinib monotherapy. Response assessment was based on criteria for BNS from the 8th International Workshop for WM. Survival from from BNS diagnosis (BNS survival), survival from ibrutinib initiation to last follow-up or death (ibrutinib survival) and time from ibrutinib initiation to ibrutinib discontinuation for toxicity, progression or death (event-free survival, EFS) were estimated. Twenty-eight patients were included in our study. The median age at BNS diagnosis was 65 years. Ibrutinib was the first line of treatment for BNS in 39% of patients. Ibrutinib was administered orally at a dose of 560 mg and 420 mg once daily in 46% and 54% of patients, respectively. Symptomatic and radiologic improvements were seen in 85% and 60% of patients within 3 months of therapy. At best response, 85% of patients had improvement or resolution of BNS symptoms, 83% had improvement or resolution of radiological abnormalities, and 47% had cleared the disease in the cerebrospinal fluid. The 2-year EFS rate on ibrutinib was 80% (95% CI 58-91%), the 2-year ibrutinib survival rate was 81% (95% CI 49-94%) and the 5-year BNS survival rate was 86% (95% CI 63-95%). Ibrutinib therapy is effective in patients with BNS and should be considered as a treatment option in these patients.

  • Submitted October 10, 2018.
  • Accepted November 29, 2018.