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Table of Contents    
Year : 2014  |  Volume : 62  |  Issue : 2  |  Page : 229-230

Bing-Neel syndrome: A cerebral richter syndrome?

1 Departement of Neuro-Oncology, APHM, Timone Hospital, 13005; Departement of Hematology, Paoli Calmettes Institute, 13273, cedex9, Marseille, France
2 Departement of Hematology, Paoli Calmettes Institute, 13273, cedex9, Marseille, France
3 Departement of Neuro-Oncology, APHM, Timone Hospital, 13005, Marseille, France

Date of Submission24-Nov-2013
Date of Decision01-Jan-2014
Date of Acceptance13-Apr-2014
Date of Web Publication14-May-2014

Correspondence Address:
Emeline Tabouret
Departement of Neuro-Oncology, APHM, Timone Hospital, 13005; Departement of Hematology, Paoli Calmettes Institute, 13273, cedex9, Marseille
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.132451

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How to cite this article:
Tabouret E, Coso D, Matta M, Barrie M, Bouabdallah R, Chinot O. Bing-Neel syndrome: A cerebral richter syndrome?. Neurol India 2014;62:229-30

How to cite this URL:
Tabouret E, Coso D, Matta M, Barrie M, Bouabdallah R, Chinot O. Bing-Neel syndrome: A cerebral richter syndrome?. Neurol India [serial online] 2014 [cited 2021 May 5];62:229-30. Available from:


Bing-Neel syndrome (BNS) is a rare neurologic complication of Waldenström macroglobulinemia (WM) involving an association with WM and central nervous system infiltration by neoplastic cells. [1],[2] Classification of BNS was historically defined into two subtypes: Focal or diffuse. [3] Here we present two cases of patients with focal forms of BNS and histology of diffuse large-B cells lymphoma (DLBCL).

Case 1: In 2003, a 72-year-old woman presented with hyperproteinemia and electrophoresis monoclonal immunoglobulin M (IgM) peak of 3.97 g/l. Bone marrow biopsy (BMB) demonstrated infiltration by lymphoplasmacytic cells. It was decided to follow-up her. In July 2006, she presented with progressive cognitive disorders. In July 2007, brain magnetic resonance imaging (MRI) showed a left occipital lesion [Figure 1]. Biopsy of the lesion confirmed the CD20+ DLBCL diagnosis. Cerebrospinal fluid (CSF) protein level was 0.33 g/dL without abnormal cells. IgM peak was at 1.80 g/L. Systemic computed tomography (CT) scan was normal. Patient was treated with four cycles of high-dose methotrexate (MTX), temozolomide and prednisone and achieved clinical and radiologic complete remission (CR). In August 2009, she presented with rapid cognitive status deterioration. MRI showed tumor progression. The patient died 2 years after DLBCL diagnosis due to recurrent lymphoma.
Figure 1: Brain magnetic resonance imaging of the first case: Homogeneous lesion located in occipital horn of lateral right ventricle

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Case 2: In January 1997, a 73-year-old woman presented with asthenia and electrophoresis monoclonal IgM peak of 28 g/L. BMB confirmed WM diagnosis. She received eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone regimen. In November 1997, first relapse was treated with six cycles of chlorambucil. In May 2000, second relapse was treated with six cycles of fludarabine. In April 2003, she presented with confusion and memory disturbance, left hemiparesis and bi-temporal hemianopsia. IgM peak was at 4.9 g/L. Brain MRI showed a right frontal lesion. 1 month later, biopsy of the lesion showed DLBC. CSF analysis showed 2.35 g/L of protein with abnormal lymphoma cells. Systemic CT scan was unremarkable. The patient received high-dose MTX, lomustine, procarbazine and prednisone. 1 month later, a brain CT scan showed a progressive disease in context of neurological deterioration. The patient died 3 months after DLBCL diagnosis due to brain lymphoma.

Waldenström macroglobulinemia is characterized by lymphoplasmacytic bone marrow infiltration with IgM monoclonal gammapathy. Neurological manifestations of WM are peripheral neuropathy, myelopathy, ischemic, or hemorrhagic cerebral stroke and cerebral tumoral infiltration, called BNS. [2] It is interesting to note that both our patients presented BNS independently of WM status. Two historical forms of BNS were described: [3] the most common characterized by diffuse cerebral infiltrations of WM cells; [4] the second "tumoral form" involves the occurrence of tumor mass as analyzed on neuro-imaging. Here we propose a new histological classification of BNS: A first infiltrative form by lymphoplasmocytoid cells and a second tumoral-DLBCL form. WM is a low-grade lymphoma, which is known to be at risk for developing high-grade lymphoma in rare cases (Richter syndrome). [5] Moreover, two reported BNS histologies [3],[6] revealed the association of both tumoral populations' cells, suggesting a cerebral transformation of WM. Therefore, BNS in its "tumoral form" could be assimilated to a Richter syndrome, a new BNS entity. Therapy of BNS appeared to be extremely variable, more related to WM treatment. [7],[8] However, these therapies are not recommended for primary cerebral lymphoma for which current treatment is high-dose MTX. [5],[9] Our two patients received high-dose MTX and one of them achieved a prolonged CR.

Bing-Neel syndrome remains a rare complication of WM. It can be classified into two categories: infiltration by lymphoplasmacytic cells and cerebral DLBCL transformation. The latter form can be considered as a Richter transformation. There is no consensual treatment for BNS, but high-dose MTX-based regimen could represent an interesting option.

  References Top

1.Bing J, Neel AV. Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infection basis. Acta Med Scan 1936;88:492-506.  Back to cited text no. 1
2.Monteiro PL, Wildi E. Waldenstrom′s macroglobulinemia with lymphorproliferative changes in the central nervous system. 4 cases. Schweiz Arch Neurol Neurochir Psychiatr 1975;116:59-82.  Back to cited text no. 2
3.Imai F, Fujisawa K, Kiya N, Ninomiya T, Ogura Y, Mizoguchi Y, et al. Intracerebral infiltration by monoclonal plasmacytoid cells in Waldenstrom′s macroglobulinemia: Case report. Neurol Med Chir (Tokyo) 1995;35:575-9.  Back to cited text no. 3
4.Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH. Bing-Neel Syndrome revisited. Clin Lymphoma Myeloma 2009;9:104-6.  Back to cited text no. 4
5.Lin P, Mansoor A, Bueso-Ramos C, Hao S, Lai R, Medeiros LJ. Diffuse large B-cell lymphoma occurring in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. Clinicopathologic features of 12 cases. Am J Clin Pathol 2003;120:246-53.  Back to cited text no. 5
6.Garderet L, Baudel JL, Cervera P, Azizi L, Maury E, Guidet B, et al. ′Indolent′ Waldenstrom′s macroglobulinemia and a cerebrospinal fluid protein level of 16 g/L. Eur J Haematol 2006;77:80-2.  Back to cited text no. 6
7.Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG. Bing-Neel syndrome: A case report and systematic review of clinical manifestations, diagnosis, and treatment options. Clin Lymphoma Myeloma 2009;9:462-6.  Back to cited text no. 7
8.Dimopoulos MA, Gertz MA, Kastritis E, Garcia-Sanz R, Kimby EK, Leblond V, et al. Update on treatment recommendations from the Fourth International Workshop on Waldenstrom′s Macroglobulinemia. J Clin Oncol 2009;27:120-6.  Back to cited text no. 8
9.Ferreri AJ, Reni M, Foppoli M, Martelli M, Pangalis GA, Frezzato M, et al. High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet 2009;374:1512-20.  Back to cited text no. 9


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