Evaluation and Management of Bing-Neel Syndrome

Sarah J Schep; Josephine M I Vos; Monique C Minnema

doi:10.1016/j.hoc.2023.04.008

Evaluation and Management of Bing-Neel Syndrome

Sarah J Schep^*, Josephine M I Vos, Monique C Minnema

^*Corresponding author for this work

Research output: Contribution to journal › Review article › peer-review

Abstract

Bing-Neel syndrome is a rare manifestation of Waldenström macroglobulinemia (WM), which is caused by infiltration of the malignant lymphoplasmacytic cells in the central nervous system. Patients can present with a diverse range of neurologic symptoms, and differentiation with other comorbidities seen in WM, such as immunoglobulin M-related polyneuropathy, can be challenging. Both the rarity of this disorder and the heterogeneity of the clinical presentation often cause a significant diagnostic delay with the risk of permanent neurologic damage. This review summarizes current knowledge regarding diagnosis, treatment and prognosis of Bing-Neel syndrome.

Original language	English
Pages (from-to)	777-786
Number of pages	10
Journal	Hematology/oncology clinics of North America
Volume	37
Issue number	4
Early online date	29 May 2023
DOIs	https://doi.org/10.1016/j.hoc.2023.04.008
Publication status	Published - Aug 2023

Keywords

Bing–Neel syndrome
Central nervous system
Cerebrospinal fluid
MRI
Waldenström macroglobulinemia

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10.1016/j.hoc.2023.04.008

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title = "Evaluation and Management of Bing-Neel Syndrome",

abstract = "Bing-Neel syndrome is a rare manifestation of Waldenstr{\"o}m macroglobulinemia (WM), which is caused by infiltration of the malignant lymphoplasmacytic cells in the central nervous system. Patients can present with a diverse range of neurologic symptoms, and differentiation with other comorbidities seen in WM, such as immunoglobulin M-related polyneuropathy, can be challenging. Both the rarity of this disorder and the heterogeneity of the clinical presentation often cause a significant diagnostic delay with the risk of permanent neurologic damage. This review summarizes current knowledge regarding diagnosis, treatment and prognosis of Bing-Neel syndrome.",

keywords = "Bing–Neel syndrome, Central nervous system, Cerebrospinal fluid, MRI, Waldenstr{\"o}m macroglobulinemia",

author = "Schep, {Sarah J} and Vos, {Josephine M I} and Minnema, {Monique C}",

note = "Funding Information: S.J. Schep: nothing to disclose. M.C. Minnema: Speakers Bureau Janssen-Cilag, WebMD Global, Consultancy Janssen-Cilag, CDR-life, BMS, GSK, research funding; BeiGene; all honoraria are institutional. J.M.I. Vos: consultancy and advisory board (Sanofi), research funding (BeiGene, China & Abbvie, United States/Genmab), Speakers bureau and conference support (BMS); all honoraria are institutional.",

year = "2023",

month = aug,

doi = "10.1016/j.hoc.2023.04.008",

language = "English",

volume = "37",

pages = "777--786",

journal = "Hematology/oncology clinics of North America",

issn = "0889-8588",

publisher = "W.B. Saunders Ltd",

number = "4",

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AU - Schep, Sarah J

AU - Vos, Josephine M I

AU - Minnema, Monique C

N1 - Funding Information: S.J. Schep: nothing to disclose. M.C. Minnema: Speakers Bureau Janssen-Cilag, WebMD Global, Consultancy Janssen-Cilag, CDR-life, BMS, GSK, research funding; BeiGene; all honoraria are institutional. J.M.I. Vos: consultancy and advisory board (Sanofi), research funding (BeiGene, China & Abbvie, United States/Genmab), Speakers bureau and conference support (BMS); all honoraria are institutional.

PY - 2023/8

Y1 - 2023/8

N2 - Bing-Neel syndrome is a rare manifestation of Waldenström macroglobulinemia (WM), which is caused by infiltration of the malignant lymphoplasmacytic cells in the central nervous system. Patients can present with a diverse range of neurologic symptoms, and differentiation with other comorbidities seen in WM, such as immunoglobulin M-related polyneuropathy, can be challenging. Both the rarity of this disorder and the heterogeneity of the clinical presentation often cause a significant diagnostic delay with the risk of permanent neurologic damage. This review summarizes current knowledge regarding diagnosis, treatment and prognosis of Bing-Neel syndrome.

AB - Bing-Neel syndrome is a rare manifestation of Waldenström macroglobulinemia (WM), which is caused by infiltration of the malignant lymphoplasmacytic cells in the central nervous system. Patients can present with a diverse range of neurologic symptoms, and differentiation with other comorbidities seen in WM, such as immunoglobulin M-related polyneuropathy, can be challenging. Both the rarity of this disorder and the heterogeneity of the clinical presentation often cause a significant diagnostic delay with the risk of permanent neurologic damage. This review summarizes current knowledge regarding diagnosis, treatment and prognosis of Bing-Neel syndrome.

KW - Bing–Neel syndrome

KW - Central nervous system

KW - Cerebrospinal fluid

KW - MRI

KW - Waldenström macroglobulinemia

UR - http://www.scopus.com/inward/record.url?scp=85160743303&partnerID=8YFLogxK

U2 - 10.1016/j.hoc.2023.04.008

DO - 10.1016/j.hoc.2023.04.008

M3 - Review article

C2 - 37258354

SN - 0889-8588

VL - 37

SP - 777

EP - 786

JO - Hematology/oncology clinics of North America

JF - Hematology/oncology clinics of North America

IS - 4

ER -

Evaluation and Management of Bing-Neel Syndrome

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Keywords

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