Successful treatment with tirabrutinib for relapsed lymphoplasmacytic lymphoma complicated by Bing–Neel syndrome

Successful treatment with tirabrutinib for relapsed Bing-Neel syndrome following high-dose methotrexate and craniospinal irradiation

“…In contrast, there are several case reports of second-generation BTK inhibitors such as zanubrutinib and tirabrutinib for treatment of BNS. 7,8,18 In two previous case reports, tirabrutinib was administered as an initial treatment after the diagnosis of BNS. 7,8 The current case is the first report of tirabrutinib for relapse of BNS after R-MPV and craniospinal irradiation, and a rapid response was observed without any adverse events.…”

“…7,8,18 In two previous case reports, tirabrutinib was administered as an initial treatment after the diagnosis of BNS. 7,8 The current case is the first report of tirabrutinib for relapse of BNS after R-MPV and craniospinal irradiation, and a rapid response was observed without any adverse events. The current case There is diffuse proliferation of small abnormal lymphocytes with plasmacytoid differentiation.…”

“…1,4 Tirabrutinib is a second-generation BTK inhibitor that has higher BTK selectivity than ibrutinib, and was approved in Japan for the treatment of LPL/ WM in August 2020. 5,6 Following approval, there have been few case reports of successful treatment of BNS using tirabrutinib, 7,8 due to the rarity of the disease. In addition, there have been no reports regarding its efficacy for relapse of BNS as tirabrutinib was administered early after the diagnosis of BNS in cases reported previously.…”

The Use of Bruton Tyrosine Kinase Inhibitors in Waldenström’s Macroglobulinemia

“…A multicenter cohort study of 28 BNS patients treated with ibrutinib demonstrated a 5-year OS rate of 86% [ 65 ]. Other BTKi are used less frequently, with case reports showing some efficacy of zanubrutinib and tirabrutinib [ 66 , 67 ].…”

The Impact of Tirabrutinib Monotherapy for Bing-Neel Syndrome in Waldenström's Macroglobulinemia

“…A phase II study of tirabrutinib monotherapy for WM demonstrated durable efficacy with an acceptable safety profile ( 13 , 15 ). Furthermore, reports by Oyama et al ( 1 ) and Saburi et al ( 16 ) have suggested that tirabrutinib monotherapy might be an effective treatment for BNS with low toxicity.…”