Bing–Neel syndrome in a patient with Waldenström Macroglobulinemia. Case report and literature review

Background. In 1936, a condition now known as BNS was first described. It is characterized by the infiltration of LPLs into the CNS, causing neurological symptoms. This rare complication occurs in approximately 1% of patients with WM. Diagnosing BNS is challenging due to its heterogeneous symptoms and requires specific tests for confirmation. Treatment for BNS is personalized and often involves high-dose chemotherapy and possibly stem cell transplantation, with early diagnosis being crucial to prevent irreversible damage. Case report. A 71-year-old male with a 20-year history of stage IV WM (MYD88 positive) presented in October 2023 with serological disease progression but no evidence of CRAB symptoms or hyperviscosity syndrome. He had a fever of up to 39°C. Antibiotic treatment improved his infectious condition. However, the patient later developed neurological symptoms, and MRI and lumbar puncture confirmed the diagnosis of BNS. Ibrutinib was initiated, and the patient's condition improved. Despite this, the patient's condition worsened, and he was transferred to supportive care. Conclusions. BNS is a rare but serious complication of WM, requiring early recognition and prompt treatment. In this case, ibrutinib played a crucial role in managing BNS symptoms. Despite progress, better treatments and more effective management strategies are still needed.