How we manage Bing-Neel syndrome
Jorge J Castillo1, Steven P Treon1
1Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
British Journal of Haematology
|August 21, 2019
Summary
Bing-Neel syndrome (BNS), a rare central nervous system complication of Waldenström macroglobulinaemia, involves WM cells infiltrating the brain. Diagnosis requires cerebrospinal fluid analysis and MRI, with emerging treatments like ibrutinib showing promise.
Area of Science:
- Neurology
- Hematology
- Oncology
Background:
- Bing-Neel syndrome (BNS) is a rare neurological complication of Waldenström macroglobulinaemia (WM), affecting approximately 1% of patients.
- BNS arises when WM cells infiltrate the central nervous system (CNS), leading to significant neurological deficits.
Purpose of the Study:
- This review aims to consolidate current knowledge on BNS.
- It covers clinical and pathological features, diagnostic criteria, and therapeutic strategies.
Main Methods:
- Diagnosis is supported by neuroimaging, particularly MRI showing leptomeningeal enhancement.
- Confirmation involves detecting clonal lymphoplasmacytic cells and the MYD88 L265P mutation in cerebrospinal fluid.
Main Results:
- Traditional treatments include CNS-penetrant chemotherapy agents like fludarabine, methotrexate, and cytarabine.
- Emerging data highlight the therapeutic potential of the Bruton Tyrosine Kinase inhibitor ibrutinib due to its CNS penetration.
Conclusions:
- Effective management of BNS necessitates therapies with robust CNS penetration.
- Ibrutinib represents a promising novel therapeutic option for patients with Bing-Neel syndrome.
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