title: [IgG4-related disease and clonal B-cell lymphoid proliferation: Description of two clinical cases and a review of literature].
abstract: IgG4-related disease (IgG4-RD) is a systemic immune-related disease that may involve the pancreas, liver, retroperitoneal space, biliary tract, salivary and lacrimal glands, eye socket, lung, and kidney. In term of pathomorphogenesis, it is a fibroinflammatory disease manifesting as a tumor-like lesion of organs, elevated serum IgG4 levels, and a morphofunctional substrate - the development of marked fibrosis and lymphoplasmacytic infiltration in the tissues with the high content of IgG4-positive plasma cells. The detection of a tumor-like nodule frequently leads to that the patients with IgG4-RD undergo major traumatic surgery for presumed cancer. At the same time, a number of investigations show the association of IgG4-RD with the development of cancer and lymphoproliferative diseases. The paper describes two clinical cases: Russia's first diagnosis of MALT lymphoma of the lacrimal gland, IgG4-positive and IgG4-RD with a rare onset with a destruction focus in the cervical vertebrae, multiple organ dysfunction, B-cell clonality in salivary gland tissue and PIgMκ secretion. It also reviews world literature on the development of lymphoproliferative diseases in the presence of IgG4-RD.
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: E V
authors.initials: EV
authors.lastname: Sokol
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: V I
authors.initials: VI
authors.lastname: Vasilyev
authors.affiliation: Hematology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: A M
authors.initials: AM
authors.lastname: Kovrigina
authors.affiliation: Helmholtz Research Institute of Eye Diseases, Ministry of Health of Russia, Moscow, Russia.
authors.forename: T N
authors.initials: TN
authors.lastname: Safonova
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: E L
authors.initials: EL
authors.lastname: Nasonov
mesh_terms: D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000276:immunology / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G