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IgG4‐related disease: Historical overview and pathology of hematological disorders
Author(s) -
Sato Yasuharu,
Notohara Kenji,
Kojima Masaru,
Takata Katsuyoshi,
Masaki Yasufumi,
Yoshino Tadashi
Publication year - 2010
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/j.1440-1827.2010.02524.x
Subject(s) - pathology , medicine , autoimmune pancreatitis , lymphoma , lymphoplasmacytic lymphoma , disease , igg4 related disease , systemic disease , lymph node , lymphatic system , waldenstrom macroglobulinemia
IgG4‐related diseases comprise a recently recognized systemic syndrome characterized by mass‐forming lesions in mainly exocrine tissue that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4‐positive plasma cells in the affected tissues, and the serum IgG4 level is increased in these patients. The present study describes the history, autoimmune pancreatitis (AIP), IgG4‐related lymphadenopathy and lymphomagenesis based upon ocular adnexal IgG4‐related disease. Lymphoplasmacytic sclerosing pancreatitis, a prototypal histological type of AIP, is now recognized as a systemic IgG4‐related disease. Lymph node lesions can be subdivided into at least five histological subtypes, and systemic IgG4‐related lymphadenopathy should be distinguished from multicentric Castleman's disease. Interleukin‐6 and CRP levels are abnormally high in multicentric Castleman's disease, but are normal in the majority of systemic IgG4‐related lymphadenopathy. Ocular adnexal IgG4‐related disease frequently involves bilateral lacrimal glands swelling, and obliterative phlebitis is rare. Moreover, some malignant lymphomas, especially mucosa‐associated lymphoid tissue lymphoma, arise from ocular adnexal IgG4‐related disease. In addition, IgG4‐producing lymphoma also exists.