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Abstract Details

Leptomeningeal Carcinomatosis in a patient with Waldenstrom Macroglobulinemia.
Neuro-oncology
P2 - Poster Session 2 (5:30 PM-6:30 PM)
7-004
A case report of Bing-Neel syndrome (BNS) in a patient with Waldenstrom Macroglobulinemia (WM).

WM is a lymphoplasmacytic lymphoma associated with IgM paraproteinemia that encompasses various neurological complications commonly appendicular/gait ataxia and cranial neuropathies. Lymphoplasmacytic cell infiltration in the central nervous system known as Bing-Neel syndrome is relatively a rare complication. From 1995-2014 there has been only 44 cases of BNS documented and only a handful of cases since then.


A 68 year old female diagnosed with Waldenstrom Macroglobulinemia in 2010, bone marrow was positive for MYD88-L265P mutation. She was in remission after last chemotherapy in 2017. She presented on 10-04-2018 with gait ataxia, worsening lower back pain, neck pain, headaches, transient muffled hearing, and hand tremors. On examination she had papilledema, hand tremors and ataxic gait.

Serum viscosity was 1.2 centipoises, serum immunofixation revealed IgM of 1898 mg/dl, normal IgA and IgG titers. Initial CSF analysis revealed protein of 366 mg/dl, WBC of 416/ cu mm

(92% lymphocytes), elevated IgA (3.2mg/dl) and IgM titers (>25mg/dl). A second CSF analysis showed opening pressure of 20 mmHg, WBC 82/ cu mm (95% Lymphocytes). CSF cytometry showed kappa restricted B cells (CD5- CD10+ CD19+ CD 20+ CD22+ CD38+ and CD45+). CSF sample was inadequate for CSF immunofixation. MRI with gadolinium showed abnormal contrast enhancement within the internal auditory canals, trigeminal nerves, along the cisternal segments of the oculomotor nerves, and extraocular muscles strongly suggestive of leptomeningeal carcinomatosis. After 2 doses of intrathecal methotrexate and chemotherapy (obinutuzumab and bendamustine), all symptoms resolved.

Practitioners should remain vigilant of BNS in a patient with WD. Systematic approach with CSF cytometry to assess immunophenotypic profile and MRI of the brain and spinal axis with gadolinium is useful. Favorable outcomes are seen after systemic/intrathecal chemotherapy, stem-cell transplantation, and purine analogs in treatment of BNS.
Authors/Disclosures
Sania Atta, MBBS (Columbia Medical Center-NYP)
PRESENTER
No disclosure on file
Adil Iqbal, MD No disclosure on file
Brij M. Ahluwalia-Singh, MD, FAAN (Westchester Medical Center-Advanced Physician Services) No disclosure on file