First-line ibrutinib for Bing-Neel syndrome
Aaron Tallant1, Daniel Selig1, Sam O Wanko1
1Walter Reed National Military Medical Center, Bethesda, Maryland, USA.
BMJ Case Reports
|October 4, 2018
Summary
This case study highlights Bing-Neel syndrome, a rare complication of Waldenstrom macroglobulinaemia. Treatment with ibrutinib effectively improved neurological symptoms and reduced disease markers.
Area of Science:
- Neurology
- Hematology
- Oncology
Background:
- Waldenstrom macroglobulinaemia is a rare lymphoproliferative disorder.
- Bing-Neel syndrome is a rare neurological complication characterized by lymphoplasmacytic infiltration of the central nervous system.
Observation:
- An elderly male patient with Waldenstrom macroglobulinaemia in remission presented with progressive gait abnormalities and falls.
- Neurological examination revealed bilateral lower extremity weakness and an unsteady gait.
- Brain and spinal MRI demonstrated leptomeningeal enhancement.
Findings:
- Cerebrospinal fluid flow cytometry confirmed a monoclonal B-cell population, diagnosing Bing-Neel syndrome.
- Treatment with ibrutinib, a Bruton's tyrosine kinase inhibitor, led to significant improvement in neurological symptoms.
- Follow-up imaging showed reduced leptomeningeal enhancement, and the patient maintained a clinical response.
Implications:
- This case underscores the importance of considering Bing-Neel syndrome in patients with Waldenstrom macroglobulinaemia presenting with neurological deficits.
- Ibrutinib demonstrates efficacy in treating Bing-Neel syndrome, offering a targeted therapeutic option.
- Early diagnosis and treatment with ibrutinib can lead to sustained clinical improvement and better outcomes.
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