IgG4-related disease and clonal B-cell lymphoid proliferation: Description of two clinical cases and a review of literature

• Main Authors: E V Sokol, V I Vasilyev, A M Kovrigina, T N Safonova, E L Nasonov
• Format: Article
• Language: Russian
• Published: "Consilium Medicum" Publishing house 2015-12-01
• Series: Терапевтический архив
• Subjects: igg4-related disease; lymphoproliferative diseases; malt lymphoma; lacrimal gland; clonality; bone foci
• Online Access: https://ter-arkhiv.ru/0040-3660/article/view/31883
• ISSN: 0040-3660; 2309-5342

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.