Diagnosing, Imaging and Successfully Treating a Debilitating Case of Bing-Neel Syndrome: A Multidisciplinary Feat

Research Article

Ann Hematol Onco. 2023; 10(1): 1417.

Diagnosing, Imaging and Successfully Treating a Debilitating Case of Bing-Neel Syndrome: A Multidisciplinary Feat

Kerley RN1,2*, O’Donnell N², Lynott F², Mulcahy R² and Hennessy B²

¹Cork University Hospital, Wilton, Cork, Ireland

²University Hospital Waterford, Dunmore Road, Waterford, Ireland

*Corresponding author: Robert N KerleyCork University Hospital, Wilton, Cork, & University Hospital Waterford, Dunmore Road, Waterford, Ireland

Received: January 10, 2023; Accepted: February 17, 2023; Published: February 24, 2023


Waldenstroms Macroglobulinaemia (WM) is a rare B-cell lymphoma representing ~2% of all haematological malignancies. While most neurological complications of WM are secondary to the overproduction of Immunoglobulin M (IgM), Bing-Neel Syndrome (BNS) is an extremely rare direct Central Nervous System (CNS) infiltration by malignant Lymphoplasmocytic Lymphoma (LPL) cells. Limited information on BNS exists in the literature with sparse case reports and case series. Here we present a diagnostically challenging BNS case successfully treated with systemic chemoimmunotherapy and ibrutinib, with remarkable clinical response.


A 63-year-old lady presented to the Emergency Department (ED) with a one-day history of binocular diplopia and evidence of abducens nerve palsy on examination. Medical history included dyslipidaemia, non-alcoholic fatty liver disease (NASH) and colonic poly removal. Visual symptoms occurred gradually over weeks without diurnal variation. Fundal examination was unremarkable. Admission bloods, chest x-ray and CT brain did not reveal a cause for the patient’s neurological symptoms. Full blood count showed haemoglobin (Hb) of 10.6 g/dL but was otherwise unremarkable. Her vasculitic, viral, and lyme serology screen were negative. Incidentally, her serum protein electrophoresis showed an IgM paraproteinemia, with a paraprotein level of 4.2 g/L. Renal function, serum calcium, beta-2-micorglobulin and plasma viscosity levels were within normal ranges. An MRI Brain, orbits and cervical cord revealed significant cervical spondylosis with multi-level nerve root impingement. Lumbar Puncture (LP) showed raised Cerebrospinal Fluid (CSF) protein (0.97 g/dL) and leukocyte count (31 x 109/L, 90% lymphocytes, 10% polymorphs). She was discharged with an incidental diagnosis of Monoclonal Gammopathy of Undetermined Significance (MGUS) with close follow from neurology and general medicine.

Progression and Diagnosis

Two weeks later the patient was re-admitted with ataxia, gait disturbance and loss of power bilaterally in her lower limbs. On examination, power was 3/5 in both lower limbs and 5/5 in her upper limbs. Tone was normal, sensation and reflexes were intact without evidence of coordination deficits and an extensor plantar reflex. A repeat MRI Brain and whole spine with contrast was negative for leptomeningeal or parenchymal enhancement. Without a diagnosis and progressive neurological deficits, a broader differential was conducted to include nerve conduction studies, a repeat autoimmune, vasculitic and viral screen, CT thorax, abdomen & pelvis, and serum ACE all of which were non-diagnostic. Finally, a repeat LPwith CSF immunophenotyping and second MRI whole spine with contrast were ordered to exclude infiltrative disease (Figure 1). This MRI result was discussed at three major neuroradiology sites in our country to form a consensus on the presence of infiltrative disease. Subtle posterior meningeal enhancement involving the distal cord at T10 and T11 vertebral bodies was observed. In the context of macroglobulinaemia, a bone marrow biopsy was performed which showed a normocellular specimen with no evidence of lymphomatous infiltration. While awaiting her CSF sample to return from flow cytometry she deteriorated with ascending paralysis compromising respiratory drive and requiring transfer to the Intensive Care Unit (ICU). The following morning, CSF immunphenotyping revealed a clonal population of kappa light chains restricted B cells, which were CD5 and CD10 negative, consistent with CNS involvement by lymphoma, in keeping with a diagnosis of BNS.

Citation: Kerley RN, O’Donnell N, Lynott F, Mulcahy R, Hennessy B. Diagnosing, Imaging and Successfully Treating a Debilitating Case of Bing-Neel Syndrome: A Multidisciplinary Feat. Ann Hematol Onco. 2023; 10(1): 1417.