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Haematologica. 2017 Jan;102(1):43-51. doi: 10.3324/haematol.2016.147728. Epub 2016 Oct 6.

Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome.

Author information

1
Department of Hematology, UMC Utrecht Cancer Center, the Netherlands m.c.minnema@umcutrecht.nl.
2
Hematology Center, Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
3
Cancer Division, University College London Hospitals NHS Foundation Trust, UK.
4
Department of Oncology and Hematology, Hôpital Universitaires de Strasbourg and Université de Strasbourg, France.
5
Service d'Hématologie-Immunologie-Cytogénétique, Centre Hospitalier de Valenciennes/Laboratoire d'Hématologie, Centre de Biologie et Pathologie, CHRU de Lille/INSERM, France.
6
Department of Neurology & Neurosurgery, Brain Center Rudolf Magnus, UMC Utrecht, The Netherlands.
7
Department of Clinical Therapeutics, National and Kapodistrian University of Athens, Greece.
8
Pôle d'Imagerie-Neuroradiologie, Hôpital de Hautepierre/CHU Strasbourg, France.
9
Laboratory of Hematology, Hôpital Pitié Salpêtrière, Paris, France.
10
Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
11
Bing Center for Waldenstrom's Macroglobulinemia, Dana Farber Cancer Institute and Harvard Medical; School, Boston, MA, USA.
12
AP-HP Hôpital Pitié Salpêtrière, UPMC univ Paris, France.
13
Department of Hematology, Academic Medical Center, Amsterdam, the Netherlands.

Abstract

Bing Neel syndrome is a rare disease manifestation of Waldenström's macroglobulinemia that results from infiltration of the central nervous system by malignant lymphoplasmacytic cells. In this guideline we describe the clinical symptoms, as well as the appropriate laboratory and radiological studies, that can aid in the diagnosis. The presentation of Bing Neel syndrome may be very diverse, and includes headaches, cognitive deficits, paresis, and psychiatric symptoms. The syndrome can present in patients with known Waldenström's macroglobulinemia, even in the absence of systemic progression, but also in previously undiagnosed patients. Diagnostic work-up should include cerebral spinal fluid analysis with multiparameter flow cytometry to establish B-cell clonality, protein electrophoresis and immunofixation for the detection and classification of a monoclonal protein as well as molecular diagnostic testing for immunoglobulin gene rearrangement and mutated MYD88. MRI of the brain and spinal cord is also essential. The second challenge is to expand our knowledge of prognosis and treatment outcome. Prospective clinical trials on Bing Neel syndrome patients that employ uniform treatment along with appropriate laboratory cerebral spinal fluid assessments and standardized MRI protocols will be invaluable, constituting a significant step forward in delineating treatment outcome for this intriguing disease manifestation.

PMID:
27758817
PMCID:
PMC5210231
DOI:
10.3324/haematol.2016.147728
[Indexed for MEDLINE]
Free PMC Article

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