A Case of Bing–Neel Syndrome Successfully Treated with Ibrutinib

Bing–Neel syndrome is a rare manifestation of Waldenström macroglobulinemia characterized by lymphoplasmacytic cells’ infiltration into the central nervous system. We present a case of a 74-year-old patient with a known diagnosis of Waldenström macroglobulinemia and newly depressed consciousness. Fl...

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Main Authors: Daniel S. O’Neil, Mark A. Francescone, Karen Khan, Alobeid Bachir, Owen A. O’Connor, Ahmed Sawas
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2018/8573105
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author Daniel S. O’Neil
Mark A. Francescone
Karen Khan
Alobeid Bachir
Owen A. O’Connor
Ahmed Sawas
author_facet Daniel S. O’Neil
Mark A. Francescone
Karen Khan
Alobeid Bachir
Owen A. O’Connor
Ahmed Sawas
author_sort Daniel S. O’Neil
collection DOAJ
description Bing–Neel syndrome is a rare manifestation of Waldenström macroglobulinemia characterized by lymphoplasmacytic cells’ infiltration into the central nervous system. We present a case of a 74-year-old patient with a known diagnosis of Waldenström macroglobulinemia and newly depressed consciousness. Flow cytology of his cerebral spinal fluid demonstrated a lambda light chain-restricted population of B-cells consistent with a CD5+ CD10+ B-cell lymphoma. Magnetic resonance imaging suggested involvement of the left optic nerve sheath and the bilateral orbital and parietal parenchyma and leptomeninges. He was diagnosed with Bing–Neel syndrome and treated with intrathecal liposomal cytarabine, intravenous high-dose methotrexate, and rituximab without improvement. Subsequently, he started treatment with ibrutinib 560 mg daily and concurrent rituximab. Within three months, he showed clinical and radiologic improvement. The patient has continued on ibrutinib and has now been stable for over 36 months. This represents the longest reported period of successful treatment of Bing–Neel syndrome with ibrutinib.
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spelling doaj-art-15e9e07aefe74a058ec5dbedfb0621ba2025-02-03T06:11:11ZengWileyCase Reports in Hematology2090-65602090-65792018-01-01201810.1155/2018/85731058573105A Case of Bing–Neel Syndrome Successfully Treated with IbrutinibDaniel S. O’Neil0Mark A. Francescone1Karen Khan2Alobeid Bachir3Owen A. O’Connor4Ahmed Sawas5Herbert Irving Comprehensive Cancer Center, Columbia University Medical Center-College of Physicians and Surgeons, New York, NY, USADepartment of Radiology, Columbia University Medical Center-College of Physicians and Surgeons, New York, NY, USACenter for Lymphoid Malignancies, Columbia University Medical Center-College of Physicians and Surgeons, New York, NY, USADepartment of Pathology and Cell Biology, Columbia University Medical Center-College of Physicians and Surgeons, New York, NY, USACenter for Lymphoid Malignancies, Columbia University Medical Center-College of Physicians and Surgeons, New York, NY, USACenter for Lymphoid Malignancies, Columbia University Medical Center-College of Physicians and Surgeons, New York, NY, USABing–Neel syndrome is a rare manifestation of Waldenström macroglobulinemia characterized by lymphoplasmacytic cells’ infiltration into the central nervous system. We present a case of a 74-year-old patient with a known diagnosis of Waldenström macroglobulinemia and newly depressed consciousness. Flow cytology of his cerebral spinal fluid demonstrated a lambda light chain-restricted population of B-cells consistent with a CD5+ CD10+ B-cell lymphoma. Magnetic resonance imaging suggested involvement of the left optic nerve sheath and the bilateral orbital and parietal parenchyma and leptomeninges. He was diagnosed with Bing–Neel syndrome and treated with intrathecal liposomal cytarabine, intravenous high-dose methotrexate, and rituximab without improvement. Subsequently, he started treatment with ibrutinib 560 mg daily and concurrent rituximab. Within three months, he showed clinical and radiologic improvement. The patient has continued on ibrutinib and has now been stable for over 36 months. This represents the longest reported period of successful treatment of Bing–Neel syndrome with ibrutinib.http://dx.doi.org/10.1155/2018/8573105
spellingShingle Daniel S. O’Neil
Mark A. Francescone
Karen Khan
Alobeid Bachir
Owen A. O’Connor
Ahmed Sawas
A Case of Bing–Neel Syndrome Successfully Treated with Ibrutinib
Case Reports in Hematology
title A Case of Bing–Neel Syndrome Successfully Treated with Ibrutinib
title_full A Case of Bing–Neel Syndrome Successfully Treated with Ibrutinib
title_fullStr A Case of Bing–Neel Syndrome Successfully Treated with Ibrutinib
title_full_unstemmed A Case of Bing–Neel Syndrome Successfully Treated with Ibrutinib
title_short A Case of Bing–Neel Syndrome Successfully Treated with Ibrutinib
title_sort case of bing neel syndrome successfully treated with ibrutinib
url http://dx.doi.org/10.1155/2018/8573105
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