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, Steven P. Treon

Research output: Contribution to journalArticle

Abstract

Ibrutinib was administered to 28 patients with BNS.Ibrutinib showed rapid and durable symptomatic and radiologic responses in patients with BNS.The treatment of patients with Bing-Neel syndrome (BNS) is not standardized. We included patients with Waldenström macroglobulinemia (WM) and a radiologic and/or cytologic diagnosis of BNS treated with ibrutinib monotherapy. Response assessment was based on criteria for BNS from the 8th International Workshop for WM. Survival 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 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 radiologic abnormalities, and 47% had cleared the disease in the cerebrospinal fluid. The 2-year EFS rate with ibrutinib was 80% (95% confidence interval [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.
Original languageEnglish
JournalBlood
Volume133
Issue number4
DOIs
Publication statusPublished - Jan 24 2019

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Multicenter Studies
Therapeutics
Waldenstrom Macroglobulinemia
Survival
Survival Rate
Confidence Intervals
Disease-Free Survival
PCI 32765
Cerebrospinal Fluid
Education

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Castillo, J. J., Itchaki, G., Paludo, J., Varettoni, M., Buske, C., Eyre, T. A., ... Treon, S. P. (2019). Ibrutinib for the treatment of Bing-Neel syndrome: a multicenter study. Blood, 133(4). https://doi.org/10.1182/blood-2018-10-879593

Ibrutinib for the treatment of Bing-Neel syndrome: a multicenter study. / Castillo, Jorge J.; Itchaki, Gilad; Paludo, Jonas; Varettoni, Marzia; Buske, Christian; Eyre, Toby A.; Chavez, Julio C.; Shain, Kenneth H.; Issa, Samar; Palomba, M. Lia; Pasvolsky, Oren; Simpson, David; Talaulikar, Dipti; Tam, Constantine S.; Tedeschi, Alessandra; Ansell, Stephen M.; Nayak, Lakshmi; Treon, Steven P.

In: Blood, Vol. 133, No. 4, 24.01.2019.

Research output: Contribution to journalArticle

Castillo, JJ, Itchaki, G, Paludo, J, Varettoni, M, Buske, C, Eyre, TA, Chavez, JC, Shain, KH, Issa, S, Palomba, ML, Pasvolsky, O, Simpson, D, Talaulikar, D, Tam, CS, Tedeschi, A, Ansell, SM, Nayak, L & Treon, SP 2019, 'Ibrutinib for the treatment of Bing-Neel syndrome: a multicenter study', Blood, vol. 133, no. 4. https://doi.org/10.1182/blood-2018-10-879593
Castillo, Jorge J. ; Itchaki, Gilad ; Paludo, Jonas ; Varettoni, Marzia ; Buske, Christian ; Eyre, Toby A. ; Chavez, Julio C. ; Shain, Kenneth H. ; Issa, Samar ; Palomba, M. Lia ; Pasvolsky, Oren ; Simpson, David ; Talaulikar, Dipti ; Tam, Constantine S. ; Tedeschi, Alessandra ; Ansell, Stephen M. ; Nayak, Lakshmi ; Treon, Steven P. / Ibrutinib for the treatment of Bing-Neel syndrome: a multicenter study. In: Blood. 2019 ; Vol. 133, No. 4.
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abstract = "Ibrutinib was administered to 28 patients with BNS.Ibrutinib showed rapid and durable symptomatic and radiologic responses in patients with BNS.The treatment of patients with Bing-Neel syndrome (BNS) is not standardized. We included patients with Waldenstr{\"o}m macroglobulinemia (WM) and a radiologic and/or cytologic diagnosis of BNS treated with ibrutinib monotherapy. Response assessment was based on criteria for BNS from the 8th International Workshop for WM. Survival 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 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 radiologic abnormalities, and 47{\%} had cleared the disease in the cerebrospinal fluid. The 2-year EFS rate with ibrutinib was 80{\%} (95{\%} confidence interval [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.",
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AU - Castillo, Jorge J.

AU - Itchaki, Gilad

AU - Paludo, Jonas

AU - Varettoni, Marzia

AU - Buske, Christian

AU - Eyre, Toby A.

AU - Chavez, Julio C.

AU - Shain, Kenneth H.

AU - Issa, Samar

AU - Palomba, M. Lia

AU - Pasvolsky, Oren

AU - Simpson, David

AU - Talaulikar, Dipti

AU - Tam, Constantine S.

AU - Tedeschi, Alessandra

AU - Ansell, Stephen M.

AU - Nayak, Lakshmi

AU - Treon, Steven P.

PY - 2019/1/24

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N2 - Ibrutinib was administered to 28 patients with BNS.Ibrutinib showed rapid and durable symptomatic and radiologic responses in patients with BNS.The treatment of patients with Bing-Neel syndrome (BNS) is not standardized. We included patients with Waldenström macroglobulinemia (WM) and a radiologic and/or cytologic diagnosis of BNS treated with ibrutinib monotherapy. Response assessment was based on criteria for BNS from the 8th International Workshop for WM. Survival 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 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 radiologic abnormalities, and 47% had cleared the disease in the cerebrospinal fluid. The 2-year EFS rate with ibrutinib was 80% (95% confidence interval [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.

AB - Ibrutinib was administered to 28 patients with BNS.Ibrutinib showed rapid and durable symptomatic and radiologic responses in patients with BNS.The treatment of patients with Bing-Neel syndrome (BNS) is not standardized. We included patients with Waldenström macroglobulinemia (WM) and a radiologic and/or cytologic diagnosis of BNS treated with ibrutinib monotherapy. Response assessment was based on criteria for BNS from the 8th International Workshop for WM. Survival 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 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 radiologic abnormalities, and 47% had cleared the disease in the cerebrospinal fluid. The 2-year EFS rate with ibrutinib was 80% (95% confidence interval [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.

U2 - 10.1182/blood-2018-10-879593

DO - 10.1182/blood-2018-10-879593

M3 - Article

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JO - Blood

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SN - 0006-4971

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