Treatment of Bing–Neel syndrome with first line sequential chemoimmunotherapy

Abstract

Rationale: Bing-Neel syndrome (BNS) is a rare manifestation of Waldenström macroglobulinemia (WM) with <200 cases reported in the literature. Herein, we describe a case of newly diagnosed BNS treated with a novel therapeutic strategy.Patient concerns: A 67-year-old woman diagnosed with asymptomatic WM 3 years ago presented with gradual vision deterioration the past 3 months. Ophthalmologic examination revealed bilateral reduction in visual acuity (7/10) and bilateral optic disc swelling which was more prominent in the left eye.Diagnoses: Brain imaging revealed bilateral swelling of optic nerves extending from the retina to the optic chiasm and swelling of the left optic tract. Patchy enhancement of optic nerves was also shown upon intravenous contrast administration. Flow cytometry of the cerebrospinal fluid (CSF) revealed the presence of k-light chain restricted, monoclonal B-lymphocytes. CSF protein electrophoresis showed a monoclonal band in the gamma region and immunofixation was positive for immunoglobulin M and kappa light chain. Thus, the diagnosis of BNS was established. Interventions:The patient was initially treated with intrathecal methotrexate and systemic chemotherapy. Following 2 intrathecal methotrexate infusions, CSF flow cytometry did not detect any cells, whereas the patient reported improvement in visual acuity. Therefore, we opted to start maintenance treatment with IV rituximab and per os ibrutinib.Outcomes: Following 1 year posttreatment initiation, visual problems have resolved completely and the patient remains on hematologic and imaging complete response.Lessons: We propose a novel sequential chemoimmunotherapy approach for BNS treatment aiming both at rapid disease control and deep and durable remission with minimization of induced toxicity.

Diagnosing, imaging, and successfully treating a debilitating case of Bing–Neel syndrome: A multidisciplinary feat

“…The goal of treatment should be to (1) reverse the patient's clinical symptoms and (2) induce prolonged progression‐free survival 6 . Remission has been reported with both IT therapies and/or systemic chemotherapies 1,2,7,8 . Use of high‐dose MTX is extrapolated from data in primary CNS lymphoma while direct evidence supports the use of rituximab in BNS 3,9 .…”

“…6 Remission has been reported with both IT therapies and/or systemic chemotherapies. 1,2,7,8 Use of high-dose MTX is extrapolated from data in primary CNS lymphoma while direct evidence supports the use of rituximab in BNS. 3,9 Ibrutinib has been increasingly employed in BNS treatment as well as in other primary CNS lymphomas.…”

Beyond Monoclonal Gammopathy of Undetermined Significance, Clinical Spectrum of Immunoglobulin M Gammopathy: A Case Series with Focus on the Diagnostic and Management Challenges

“…In case three, B-cells expressed CD19, CD20, CD45, and kappa light chain. Further, case three was positive for myeloma protein (M protein) in SPEP (along with cases one and two), an abnormal protein produced by plasma cells and an essential component of Bing–Neel syndrome diagnostic workup [ 14 ], confirming plasma cell proliferation.…”

Primary central nervous system diffuse large B‐cell lymphoma masqueraded as Bing‐Neel syndrome: Steps in management and review of future directions

“…Although there is no standardized approach for treatment, therapy should begin with identifying individuals who are symptomatic from their disease and requires antineoplastic agents that have the ability to penetrate the blood-brain barrier or agents available for intrathecal administration. 3,14,15 Treating those who are asymptomatic is felt to be contraindicated since the aim of treatment is not curative and tailored more toward reversing symptoms and inducing long term progression free survival. 3,14 Previously, systemic chemotherapy with purine nucleoside analogs such as cytarabine, fludarabine, and bendamustine, known to be effective at treating other lymphoproliferative disorders such as WM, showed promise as therapy for the treatment of BNS.…”