RESUMO
It is well recognized that B-cell clonal disorders such as Waldenstrom's macroglobulinaemia may affect the central nervous system by direct infiltration of malignant B cells (Bing-Neel syndrome). However, there is no recognition in the current literature of a clear link between paraproteinaemia and primary brain tumours such as glioma. We present 3 cases of classical IgM paraproteinaemic neuropathy who developed glioblastoma in the course of their illness following treatment with chemoimmunotherapy (CIT). Due to the progressive symptomatic nature of their neuropathy, all 3 patients were treated with CIT. The patients presented with glioblastoma, IDH-wildtype at 9 months, 5 years, and 6 years following treatment completion. None of the patients had unequivocal evidence of known predisposing factors for glioblastoma. Both disorders are exceedingly rare and the chance of random association is less than one in a million. Potential common pathogenic mechanisms include the influence of paraproteins and circulating lymphoplasmacytic cells on blood-brain permeability and CNS immune micro-environment as well as raised circulating angiogenic cytokines such as vascular endothelial growth factor. In cases with anti-myelin-associated glycoprotein (MAG) antibodies, surface MAG on glial cells may act as a target releasing cells from growth inhibition. We suggest that all glioblastoma cases be screened at diagnosis for serum paraproteins and that such cases be reported to central registries to establish the frequency of the association more accurately.
Assuntos
Ciclofosfamida , Doenças do Sistema Nervoso Periférico , Prednisolona , Rituximab , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/uso terapêutico , Humanos , Imunoglobulina M , Imunoterapia , Doenças do Sistema Nervoso Periférico/terapia , Prednisolona/uso terapêutico , Rituximab/uso terapêutico , Resultado do TratamentoRESUMO
Chronic inflammatory demyelinating polyneuropathy (CIDP) has a variable clinical course with 15% remaining refractory to treatment. We report a woman with severe refractory CIDP and coexisting chronic lymphocytic leukaemia (CLL) who improved dramatically after chemoimmunotherapy appropriate for the CLL, including rituximab. A subsequent CIDP relapse after 15 months responded again to similar treatment, and the improvement has been maintained with 3-monthly rituximab infusions as sole ongoing therapy. The case suggests that CIDP refractory to conventional treatment may have associated pathology, in this case haematological malignancy, and that treating the malignancy can effectively treat the CIDP.