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 Maxted Ansell, Lakshmi Nayak, Steven P. Treon

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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 fromibrutinib initiation to ibrutinib discontinuation for toxicity, progression, or death (event-free survival [EFS]) were estimated. Twenty-eight patients were included in our study. Themedian age atBNS 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 ibrutinibwas 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 ratewas 86% (95% CI, 63%-95%). Ibrutinib therapy is effectiveinpatientswithBNSandshouldbeconsideredas atreatmentoptioninthese patients.

Original languageEnglish (US)
Pages (from-to)299-305
Number of pages7
JournalBlood
Volume133
Issue number4
DOIs
StatePublished - Jan 24 2019

Fingerprint

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

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

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), 299-305. 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 Maxted; Nayak, Lakshmi; Treon, Steven P.

In: Blood, Vol. 133, No. 4, 24.01.2019, p. 299-305.

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, pp. 299-305. https://doi.org/10.1182/blood-2018-10-879593
Castillo JJ, Itchaki G, Paludo J, Varettoni M, Buske C, Eyre TA et al. Ibrutinib for the treatment of Bing-Neel syndrome: A multicenter study. Blood. 2019 Jan 24;133(4):299-305. 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 Maxted ; Nayak, Lakshmi ; Treon, Steven P. / Ibrutinib for the treatment of Bing-Neel syndrome : A multicenter study. In: Blood. 2019 ; Vol. 133, No. 4. pp. 299-305.
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