title: Distinct lymphocytes subsets in IgM-related neuropathy: clinical-immunological correlations.
abstract: IgM-related neuropathy generally presents as a late-onset demyelinating polyneuropathy with predominant sensory loss and ataxia. However, we recently reported the clinical, neurophysiological and pathological findings from our cohort and identified in about a third of patients an atypical phenotype. We analyzed by flow cytometry the different lymphocytes subsets in the peripheral blood of patients affected by IgM-related neuropathy, chronic inflammatory demyelinating polyneuropathy (CIDP), monoclonal gammopathy of undetermined significance and healthy subjects, to investigate whether different immunological patterns may differentiate the classical phenotype from atypical forms. IFN-gamma producing CD4+ and CD8+ T lymphocytes, as well as CD4+ and CD8+ T cells expressing T-bet (T-helper type 1, Th1) were increased in CIDP patients. The percentage of circulating CD4+ and CD8+ T cells producing IL-10 as well as the percentage of CD19+ cells expressing Blimp-1 were higher in patients with IgM-neuropathy. We did not find any significant differences in the different lymphocytes subsets in the IgM-related neuropathy between patients with classical and atypical phenotype. Th1 cells are increased in CIDP patients while a T helper type 2-phenotype seems to prevail in patients with IgM-neuropathy. Further studies involving a larger patient population are needed to evaluate if different lymphocytes subset may be involved in different clinical phenotypes of IgM-related neuropathy.
authors.affiliation: Fondazione Don Gnocchi ONLUS, Milan, Italy.
authors.forename: Raffaele
authors.initials: R
authors.lastname: Iorio
authors.forename: Mario
authors.initials: M
authors.lastname: Sabatelli
authors.forename: Alessandra
authors.initials: A
authors.lastname: Del Grande
authors.forename: Giulia
authors.initials: G
authors.lastname: Bisogni
authors.forename: Valentina
authors.initials: V
authors.lastname: Damato
authors.forename: Domenico
authors.initials: D
authors.lastname: Plantone
authors.forename: Alessandro
authors.initials: A
authors.lastname: Marti
authors.forename: Giovanni
authors.initials: G
authors.lastname: Frisullo
authors.forename: Angela
authors.initials: A
authors.lastname: Romano
authors.forename: Paolo Maria
authors.initials: PM
authors.lastname: Rossini
authors.forename: Marco
authors.initials: M
authors.lastname: Luigetti
mesh_terms: D015331:Cohort Studies; D016207:Cytokines / Q000378:metabolism; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology*; D016131:Lymphocyte Subsets / Q000166:cytology / Q000378:metabolism*; D009431:Neural Conduction; D011129:Polyradiculoneuropathy / Q000276:immunology*; D014157:Transcription Factors / Q000378:metabolism
chemical_list: D016207:Cytokines; D007075:Immunoglobulin M; D014157:Transcription Factors