J Korean Med Sci.  2007 Dec;22(6):1079-1081. 10.3346/jkms.2007.22.6.1079.

Therapeutic Experience of Bing-Neel Syndrome Associated with Waldenstrom's Macroglobulinemia

• Kim HD
• Shin KC
• Cho HS
• Kim MK
• Lee KH
• Hyun MS

• KMID: 1785774
• DOI: http://doi.org/10.3346/jkms.2007.22.6.1079

Abstract

Waldenstrom's macroglobulinemia is an uncommon low-grade B-cell lymphoproliferative disorder in which monoclonal immunoglobulin M is produced. Neurological symptoms due to hyperviscosity are frequent manifestations of Waldenstrom's macroglobulinemia. However, central nervous system infiltration by plasmacytoid lymphocytes (Bing-Neel syndrome) has only rarely been reported. We report a case of a 51-yr-old woman suffering from Waldenstrom's macroglobulinemia who complained of persistant headache. Brain magnetic resonance imaging revealed an extra-axial soft tissue mass along the left cavernous sinus, left tentorium, right tentorium, and falx cerebri. A stereotactic biopsy of dural tissue from the falx was performed and showed plasmacytoid lymphocyte infiltration. The patient became symptom- free with irradiation of the whole brain followed by chemotherapy with fludarabine.

Keyword

Waldenstrom's Macroglobulinemia; Central Nervous System

MeSH Terms

Antibodies, Monoclonal/therapeutic use
Brain/pathology
Central Nervous System Diseases/etiology/*therapy
Cranial Irradiation
Female
Humans
Middle Aged
Syndrome
Vidarabine/analogs & derivatives/therapeutic use
Waldenstrom Macroglobulinemia/*complications

Figure

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  Fig. 1 (A) Contrast enhanced axial and coronal T1-weighted images show a well-enhanced mass along the left tentorium and cavernous sinus (arrows). This mass also extends into the contralateral tentorium and falx (arrowheads). (B) Marked decrease in the size of the mass in the tentorium and falx after radiation therapy and fludarabine treatment.

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  Fig. 2 Stereostactic brain biopsy showing diffuse infiltration of atypical plasmacytoid lymphocytes into the dural fibrous tissue (A) Hematoxylin & eosin (original magnification ×200); (B) Positive immunohistochemical staining for CD20 (original magnification ×40).

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