@article {7048,
	title = {Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome},
	journal = {Haematologica},
	volume = {102},
	year = {2017},
	note = {Export Date: 18 February 2017References: Malkani, R.G., Tallman, M., Gottardi-Littell, N., Bing-Neel syndrome: An illustrative case and a comprehensive review of the published literature (2010) J Neurooncol., 96 (3), pp. 301-312;Bing, J., Von Neel, A., Two cases of hyperglobulinaemia with affection of the central nervous system on a toxi-infectious basis (1936) Acta Med Scand, 88, pp. 492-506; 
Simon, L., Fitsiori, A., Lemal, R., Bing- Neel syndrome, a rare complication of Waldenstrom macroglobulinemia: Analysis of 44 cases and review of the literature. A study on behalf of the French Innovative Leukemia Organization (FILO) (2015) Haematologica, 100 (12), pp. 1587-1594; 
Castillo, J.J., D{\textquoteright}sa, S., Lunn, M.P., Central nervous system involvement by Waldenstrom macroglobulinaemia (Bing- Neel syndrome): A multi-institutional retrospective study (2016) Br J Haematol, 172 (5), pp. 709-715; 
Fintelmann, F., Forghani, R., Schaefer, P.W., Hochberg, E.P., Hochberg, F.H., Bing-Neel Syndrome revisited (2009) Clin Lymphoma Myeloma, 9 (1), pp. 104-106; 
Ly, K.I., Fintelmann, F., Forghani, R., Schaefer, P.W., Hochberg, E.P., Hochberg, F.H., Novel diagnostic approaches in Bing-Neel syndrome (2011) Clin Lymphoma Myeloma Leuk, 11 (1), pp. 180-183; 
Stone, M.J., Bogen, S.A., Evidence-based focused review of management of hyperviscosity syndrome (2012) Blood, 119 (10), pp. 2205-2208; 
Stork, A.C., van der Pol, W.L., Franssen, H., Jacobs, B.C., Notermans, N.C., Clinical phenotype of patients with neuropathy associated with monoclonal gammopathy: A comparative study and a review of the literature (2014) J Neurol, 261 (7), pp. 1398-1404; 
Dalakas, M.C., Pathogenesis and Treatment of Anti-MAG Neuropathy (2010) Curr Treat Options Neurol, 12 (2), pp. 71-83; 
Da Silva, A.N., Lopes, M.B., Schiff, D., Rare pathological variants and presentations of primary central nervous system lymphomas (2006) Neurosurg Focus, 21 (5), pp. E7; 
Kulkarni, T., Treon, S.P., Manning, R.A., Clinical Characteristics and Treatment Outcome Of CNS Involvement (Bing-Neel Syndrome) In Waldenstroms Macroglobulinemia Blood, 122 (21), p. 5090; 
Abbi, K.K., Muzaffar, M., Gaudin, D., Booth, R.L., Jr., Feldmeier, J.J., Skeel, R.T., Primary CNS lymphoplasmacytic lymphoma: A case report and review of literature (2013) Hematol Oncol Stem Cell Ther, 6 (2), pp. 76-78; 
Owen, R.G., Treon, S.P., Al-Katib, A., Clinicopathological definition of Waldenstrom{\textquoteright}s macroglobulinemia: Consensus panel recommendations from the Second International Workshop on Waldenstrom{\textquoteright}s Macroglobulinemia (2003) Semin Oncol, 30 (2), pp. 110-115; 
Kikukawa, Y., Yamamura-Fujimoto, A., Endo, S., Successful Treatment of Bing-Neel Syndrome Accompanying Waldenstrom{\textquoteright}s Macroglobulinemia with R-MPV: A Case Report (2015) J Clin Exp Hematop, 55 (2), pp. 113-119; 
Baraniskin, A., Deckert, M., Schulte- Altedorneburg, G., Schlegel, U., Schroers, R., Current strategies in the diagnosis of diffuse large B-cell lymphoma of the central nervous system (2012) Br J Haematol, 156 (4), pp. 421-432; 
Bromberg, J.E., Breems, D.A., Kraan, J., CSF flow cytometry greatly improves diagnostic accuracy in CNS hematologic malignancies (2007) Neurology, 68 (20), pp. 1674-1679; 
Van Cauwenberge, M.G., Depreter, B., Dumoulin, E.N., Emmerechts, J., Nollet, F., Vanopdenbosch, L.J., Bing-Neel syndrome: Two unexpected cases and a review of the literature (2015) J Neurol Sci, 356 (1-2), pp. 19-26; 
Poulain, S., Boyle, E.M., Roumier, C., MYD88 L265P mutation contributes to the diagnosis of Bing Neel syndrome (2014) Br J Haematol, 167 (4), pp. 506-513; 
de Jongste, A.H., Kraan, J., van den Broek, P.D., Use of TransFix cerebrospinal fluid storage tubes prevents cellular loss and enhances flow cytometric detection of malignant hematological cells after 18 hours of storage (2014) Cytometry B Clin Cytom, 86 (4), pp. 272-279; 
Garderet, L., Baudel, J.L., Cervera, P., {\textquoteright}Indolent{\textquoteright} Waldenstrom{\textquoteright}s macroglobulinemia and a cerebrospinal fluid protein level of 16 g/L (2006) Eur J Haematol, 77 (1), pp. 80-82; 
Gupta, N., Gupta, S., Al, U.O., Pokuri, V., Hatoum, H., Bhat, S., Bing-Neel syndrome in a patient with Waldenstrom{\textquoteright}s macroglobulinemia: A challenging diagnosis in the face of normal brain imaging (2014) CNS Neurosci Ther, 20 (10), pp. 945-946; 
Zetterberg, H., Pathognomonic cerebrospinal fluid findings in Bing-Neel syndrome (2011) J Neurooncol, 104 (2), p. 615; 
Dalakas, M.C., Papadopoulos, N.M., Paraproteins in the spinal fluid of patients with paraproteinemic polyneuropathies (1984) Ann Neurol, 15 (6), pp. 590-593; 
van Krieken, J.H., Langerak, A.W., Macintyre, E.A., Improved reliability of lymphoma diagnostics via PCR-based clonality testing: Report of the BIOMED-2 Concerted Action BHM4-CT98-3936 (2007) Leukemia, 21 (2), pp. 201-206; 
Liu, L., Cao, F., Wang, S., Zhou, J., Yang, G., Wang, C., Detection of malignant B lymphocytes by PCR clonality assay using direct lysis of cerebrospinal fluid and low volume specimens (2015) Int J Lab Hematol, 37 (2), pp. 165-173; 
Frustaci, A.M., Rusconi, C., Picardi, P., Bing Neel Syndrome in a Previously Untreated Patient With Waldenstrom{\textquoteright}s Macroglobulinemia: Contribution of MYD88 L265P Mutation on Cerebrospinal Fluid (2016) Clin Lymphoma Myeloma Leuk, 16 (1), pp. e7-e9; 
Treon, S.P., Xu, L., Yang, G., MYD88 L265P somatic mutation in Waldenstrom{\textquoteright}s macroglobulinemia (2012) N Engl J Med, 367 (9), pp. 826-833; 
Treon, S.P., Xu, L., Hunter, Z., MYD88 Mutations and Response to Ibrutinib in Waldenstrom{\textquoteright}s Macroglobulinemia (2015) N Engl J Med, 373 (6), pp. 584-586; 
Xu, L., Hunter, Z.R., Yang, G., Detection of MYD88 L265P in peripheral blood of patients with Waldenstrom{\textquoteright}s Macroglobulinemia and IgM monoclonal gammopathy of undetermined significance (2014) Leukemia, 28 (8), pp. 1698-1704; 
Yamada, S., Ishida, Y., Matsuno, A., Yamazaki, K., Primary diffuse large B-cell lymphomas of central nervous system exhibit remarkably high prevalence of oncogenic MYD88 and CD79B mutations (2015) Leuk Lymphoma, 56 (7), pp. 2141-2145; 
Poulain, S., Boyle, E.M., Tricot, S., Absence of CXCR4 mutations but high incidence of double mutant in CD79A/B and MYD88 in primary central nervous system lymphoma (2015) Br J Haematol, 170 (2), pp. 285-287; 
Kraan, W., Horlings, H.M., Van, K.M., High prevalence of oncogenic MYD88 and CD79B mutations in diffuse large B-cell lymphomas presenting at immune-privileged sites (2013) Blood Cancer J, 3; 
Hunter, Z.R., Xu, L., Yang, G., The genomic landscape of Waldenstrom macroglobulinemia is characterized by highly recurring MYD88 and WHIM-like CXCR4 mutations, and small somatic deletions associated with B-cell lymphomagenesis (2014) Blood, 123 (11), pp. 1637-1646; 
Ngo, H.T., Leleu, X., Lee, J., SDF- 1/CXCR4 and VLA-4 interaction regulates homing in Waldenstrom macroglobulinemia (2008) Blood, 112 (1), pp. 150-158; 
Treon, S.P., Cao, Y., Xu, L., Yang, G., Liu, X., Hunter, Z.R., Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenstrom macroglobulinemia (2014) Blood, 123 (18), pp. 2791-2796; 
Rigamonti, A., Lauria, G., Melzi, P., A case of Bing-Neel syndrome presenting as spinal cord compression (2014) J Neurol Sci, 346 (1-2), pp. 345-347; 
Logothetis, J., Silvestein, P., Coe, J., Neurologic aspects of Waldenstrom{\textquoteright}s macroglobulinemia; report of a case (1960) Arch Neurol, 3, pp. 564-573; 
Kim, H.J., Suh, S.I., Kim, J.H., Kim, B.J., Brain magnetic resolution imaging to diagnose bingneel syndrome (2009) J Korean Neurosurg Soc, 46 (6), pp. 588-591; 
Drappatz, J., Akar, S., Fisher, D.C., Samuels, M.A., Kesari, S., Imaging of Bing-Neel syndrome (2008) Neurology, 70 (16), p. 1364; 
Rigual, D., Qiu, J., Fenstermaker, R.A., Fabiano, A.J., Tumoral Bing-Neel Syndrome presenting as a cerebellar mass (2013) Clin Neurol Neurosurg, 115 (6), pp. 823-826; 
Morel, P., Duhamel, A., Gobbi, P., International prognostic scoring system for Waldenstrom macroglobulinemia (2009) Blood, 113 (18), pp. 4163-4170; 
Kastritis, E., Kyrtsonis, M.C., Hadjiharissi, E., Validation of the International Prognostic Scoring System (IPSS) for Waldenstrom{\textquoteright}s macroglobulinemia (WM) and the importance of serum lactate dehydrogenase (LDH) (2010) Leuk Res, 34 (10), pp. 1340-1343; 
Hughes, M.S., Atkins, E.J., Cestari, D.M., Stacy, R.C., Hochberg, F., Isolated optic nerve, chiasm, and tract involvement in Bing-Neel Syndrome (2014) J Neuroophthalmol, 34 (4), pp. 340-345; 
Stacy, R.C., Jakobiec, F.A., Hochberg, F.H., Hochberg, E.P., Cestari, D.M., Orbital involvement in Bing-Neel syndrome (2010) J Neuroophthalmol, 30 (3), pp. 255-259; 
Kristinsson, S.Y., Bjorkholm, M., Landgren, O., Survival in monoclonal gammopathy of undetermined significance and Waldenstrom macroglobulinemia (2013) Clin Lymphoma Myeloma Leuk, 13 (2), pp. 187-190; 
Vos, J.M., Kersten, M.J., Kraan, W., Effective treatment of Bing-Neel Syndrome with oral fludarabine: A case series of four consecutive patients (2016) Br J Haematol, 172 (3), pp. 461-464; 
Pentsova, E., Rosenblum, M., Holodny, A., Palomba, M.L., Omuro, A., Chemotherapyrelated magnetic resonance imaging abnormalities mimicking disease progression following intraventricular liposomal cytarabine and high dose methotrexate for neurolymphomatosis (2012) Leuk Lymphoma, 53 (8), pp. 1620-1622; 
Cabannes-Hamy, A., Lemal, R., Goldwirt, L., Efficacy of ibrutinib in the treatment of Bing-Neel syndrome (2016) Am J Hematol, 91 (3), pp. E17-E19; 
Weller, M., Glucocorticoid treatment of primary CNS lymphoma (1999) J Neurooncol, 43 (3), pp. 237-239; 
Hoang-Xuan, K., Bessell, E., Bromberg, J., Diagnosis and treatment of primary CNS lymphoma in immunocompetent patients: Guidelines from the European Association for Neuro-Oncology (2015) Lancet Oncol, 16 (7), pp. e322-e332; 
Arumugam, M., Raes, J., Pelletier, E., Enterotypes of the human gut microbiome (2011) Nature, 473 (7346), pp. 174-180; 
Richards, A.I., Response of meningeal Waldenstrom{\textquoteright}s macroglobulinemia to 2- chlorodeoxyadenosine (1995) J Clin Oncol, 13 (9), p. 2476; 
Varettoni, M., Marchioni, E., Bonfichi, M., Successful treatment with Rituximab and Bendamustine in a patient with newly diagnosed Waldenstrom{\textquoteright}s Macroglobulinemia complicated by Bing- Neel syndrome (2015) Am J Hematol, 90 (8), pp. E152-E153; 
Bernard, S., Goldwirt, L., Amorim, S., Activity of ibrutinib in mantle cell lymphoma patients with central nervous system relapse (2015) Blood, 126 (14), pp. 1695-1698; 
Mason, C., Savona, S., Xu, L., Treon, S.P., Allen SL. Ibrutinib penetrates the blood brain barrier and shows efficacy in the therapy of Bing Neel syndrome (2016) Blood, , Jul 13. [Epub ahead of print]; 
Rubenstein, J.L., Fridlyand, J., Abrey, L., Phase I study of intraventricular administration of rituximab in patients with recurrent CNS and intraocular lymphoma (2007) J Clin Oncol, 25 (11), pp. 1350-1356; 
Bromberg, J.E., Doorduijn, J.K., Baars, J.W., van Imhoff, G.W., Enting, R., van den Bent, M.J., Acute painful lumbosacral paresthesia after intrathecal rituximab (2012) J Neurol, 259 (3), pp. 559-561; 
Abdallah, A.O., Atrash, S., Muzaffar, J., Successful treatment of Bing-Neel syndrome using intrathecal chemotherapy and systemic combination chemotherapy followed by BEAM auto-transplant: A case report and review of literature (2013) Clin Lymphoma Myeloma Leuk, 13 (4), pp. 502-506; 
Shimizu, K., Fujisawa, K., Yamamoto, H., Mizoguchi, Y., Hara, K., Importance of central nervous system involvement by neoplastic cells in a patient with Waldenstrom{\textquoteright}s macroglobulinemia developing neurologic abnormalities (1993) Acta Haematol, 90 (4), pp. 206-208; 
Imai, F., Fujisawa, K., Kiya, N., Intracerebral infiltration by monoclonal plasmacytoid cells in Waldenstrom{\textquoteright}s macroglobulinemia--case report (1995) Neurol Med Chir (Tokyo), 35 (8), pp. 575-579; 
Saad, S., Wang, T.J., Neurocognitive Deficits After Radiation Therapy for Brain Malignancies (2015) Am J Clin Oncol, 38 (6), pp. 634-640; 
Bhatti, M.T., Yuan, C., Winter, W., McSwain, A.S., Okun, M.S., Bilateral sixth nerve paresis in the Bing-Neel syndrome (2005) Neurology, 64 (3), pp. 576-577; 
Sanchez-Guerrero, S., Castillo, J.J., Bing-Neel syndrome: A rare complication of Waldenstrom macroglobulinemia (2015) Blood, 126 (11), p. 1390; 
Delgado, J., Canales, M.A., Garcia, B., Alvarez- Ferreira, J., Garcia-Grande, A., Hernandez- Navarro, F., Radiation therapy and combination of cladribine, cyclophosphamide, and prednisone as treatment of Bing-Neel syndrome: Case report and review of the literature (2002) Am J Hematol, 69 (2), pp. 127-131; 
Torrey, J.J., Katakkar, S.B., Treatable meningeal involvement in Waldenstrom{\textquoteright}s macroglobulinemia (1984) Ann Intern Med, 101 (3), pp. 345-347; 
Doshi, R.R., Silkiss, R.Z., Imes, R.K., Orbital involvement in Bing-Neel syndrome (2011) J Neuroophthalmol, 31 (1), pp. 94-95; 
Ritzenthaler, T., Leray, V., Bourdin, G., Ventriculitis revealing Bing-Neel syndrome in a patient without Waldenstrom{\textquoteright}s macroglobulinemia (2013) Clin Neurol Neurosurg, 115 (1), pp. 82-84; 
Morita, K., Yoshimi, A., Masuda, A., Ichikawa, M., Yatomi, Y., Kurokawa, M., Unique association of Waldenstrom macroglobulinemia with optic neuritis and monoclonal T cell expansion (2013) Int J Hematol, 98 (2), pp. 247-249; 
Jennane, S., Doghmi, K., Mahtat, E.M., Messaoudi, N., Varet, B., Mikdame, M., Bing and neel syndrome (2012) Case Rep Hematol., 2012. , 845091
},
	month = {2017},
	pages = {43 - 51},
	abstract = {Bing Neel syndrome is a rare disease manifestation of Waldenstr{\"o}m{\textquoteright}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 Waldenstr{\"o}m{\textquoteright}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. {\textcopyright} 2017 Ferrata Storti Foundation.},
	url = {https://www.scopus.com/inward/record.uri?eid=2-s2.0-85008316367\&doi=10.3324\%2fhaematol.2016.147728\&partnerID=40\&md5=6d9341016e9d44ae7fd975615a8ace19},
	author = {Minnema, M.C. and Kimby, E. and D{\textquoteright}Sa, S. and Fornecker, L.-M. and Poulain, S. and Snijders, T.J. and Kastritis, E. and Kremer, S. and Fitsiori, A. and Simon, L. and Davi, F. and Lunn, M. and Castillo, J.J. and Patterson, C.J. and Le Garff-Tavernier, M. and Costopoulos, M. and Leblond, V. and Kersten, M.-J. and Dimopoulos, M.A. and Treon, S.P.}
}
