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Nervous system lymphoid infiltration in Waldenström's macroglobulinemia. A case report
S Massengo1, L Riffaud, X Morandi
1Department of Neurology, Pontchaillou Hospital, Rennes, France.
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.
Insights
Waldenström's macroglobulinemia (Wm) can cause rare neurological issues. This case report details a patient with peripheral neuropathy and direct malignant lymphoid infiltration (MLI) of the nervous system, successfully treated with chemotherapy.
Area of Science:
- Neurology
- Hematology
- Oncology
Background:
- Waldenström's macroglobulinemia (Wm) is a rare lymphoproliferative disorder.
- Neurological complications in Wm are uncommon and their pathogenesis is debated.
- Direct malignant lymphoid infiltration (MLI) of the nervous system in Wm is exceedingly rare.
Observation:
- A 67-year-old woman with a 10-year history of asymptomatic Wm developed neurological complications.
- The patient presented with peripheral demyelinating polyneuropathy and MLI affecting the nervous system.
- MLI was localized to the lower perimedullary and cauda equina regions, with potential centromedullary involvement.
Findings:
- Non-invasive methods, including MRI and cerebrospinal fluid analysis, confirmed MLI.
- The patient received both general and intrathecal chemotherapy.
- Treatment resulted in significant clinical, biological, and radiological improvement over four years.
Implications:
- This case highlights the possibility of direct MLI in Wm, even in previously asymptomatic patients.
- Effective treatment strategies involving chemotherapy can lead to favorable outcomes.
- Further research into the mechanisms and management of neurological complications in Wm is warranted.