Abstract
Neurological complications may occur in the course of Waldenström's macroglobulinemia (Wm). These complications affect both the central and peripheral nervous system. Pathogenesis is debated and several mechanisms have been suggested. Direct malignant lymphoid infiltration (MLI) is very rare and has only been reported in a few cases.
Case report. A 67-year-old woman suffered from Wm for 10 years before she presented neurological complications of the disease. She did not receive any specific treatment for Wm owing to a lack of symptoms. The neurological complications consisted of peripheral demyelinating polyneuropathy and MLI of the nervous system. This infiltration was located in the lower perimedullary and cauda equina roots regions, with possible centromedullary involvement. Indirect demonstration of MLI was achieved through non-invasive tests including magnetic resonance imaging and detailed cerebrospinal fluid examination. General and intrathecal chemotherapy led to clinical, biological and radiological improvement over a four-year period.
Similar content being viewed by others
References
Bing J, Neel VA: Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infectious basis. Acta Med Scand 88: 492–506, 1936
Aarseth S, Osfad E, Terril A: Macroglobulinemia Waldenström. A case with haemolytic syndrome and involvement of the nervous system. Acta Med Scand 169: 691–699, 1961
Edgar R, Dutcher TF: Histopathology of the Bing-Neel syndrome. Neurology 11: 239–245, 1961
Bichel J, Bing J, Harboe N: Another case of hyperglobulinemia and affection of the central nervous system. Acta Med Scand 138: 1–14, 1950
Monteiro L, Wildi E: Macroglobulinémie de Waldenström avec atteinte lymphoprolif´erative du système nerveux central ('syndrome de Bing et Neel'). A propos de 4 cas anatomo-cliniques. Arch Suiss Neurol Neurochir Psychiatr 116: 59–82, 1975
Logothetis J, Silverstein P, Coe J: Neurologic aspects of Waldenström's macroglobulinemia: report of a case. Arch Neurol 3: 564–573, 1960
Dimopoulos MA, Galani E, Matsouka C: Waldenström's macroglobulinemia. Hematol Oncol Clin North Am 13: 1351–1366, 1999
Kyle RA: Diagnostic criteria of multiple myeloma. Hematol Oncol Clin North Am 6: 347–358, 1992
Abad S, Zagdanski AM, Brechignac S, Thioliere B, Brouet JC, Mariette X: Neurolymphomatosis in Waldenström's macroglobulinemia. Br J Haematol 106: 100–103, 1999
Shimizu K, Fujizawa K, Yamamoto H, Mizoguchi Y, Hara K: Importance of central nervous system involvement by neoplastic cells in a patient with Waldenström's macroglobulinemia develo** neurologic abnormalities. Acta Haematol 90: 206–208, 1993
Civit T, Coulbois S, Baylac F, Taillandier L, Auque J: Macroglobulinémie de Waldenström et prolifération lymphoplasmocytaire cérébrale: le syndrome de Bing et Neel. A propos d'une nouvelle observation. Neurochirurgie 43: 245–249, 1997
Delgado J, Canales MA, 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. Am J Hematol 69: 127–131, 2002
Zollinger HU: Die pathologische Anatomie der Makroglobulinämie Waldenström. Helv Med Acta 25: 153–183, 1958
Wanner VJ, Siebenmann R. Uber eine subakut verlaufende osteolytische Form der macroglobulinämie Waldenström mit plasmazellen leukämie. Schweiz Med Wschr 87: 1243–1246, 1957
Woimant F, Schanen A, Bertail MA, Dupuy M, Lecoz P, Haguenau M, Pepin B: Myélopathie révélatrice d'une macroglobulinémie. Ann Med Interne 136: 121–124, 1985
Richards AI: Response of meningeal Waldenström's macroglobulinemia to 2-chlorodeoxyadenosine. J Clin Oncol 13: 2476, 1995
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Massengo, S., Riffaud, L., Morandi, X. et al. Nervous system lymphoid infiltration in Waldenström's macroglobulinemia. A case report. J Neurooncol 62, 353–358 (2003). https://doi.org/10.1023/A:1023393532546
Issue Date:
DOI: https://doi.org/10.1023/A:1023393532546