Peripheral T-cell Lymphoma that Developed During the Follow-Up of IgG4-Related Disease

Abstract

IgG4-related disease is a recently recognized fibroinflammatory disorder characterized by extensive IgG4-positive plasma cell and lymphocyte infiltration of various organs. The pancreatic manifestation of IgG4-related disease is called autoimmune pancreatitis (AIP), in which autoimmune mechanisms are likely involved. On the other hand, some autoimmune and chronic inflammatory disorders, such as Sjögren's syndrome and rheumatoid arthritis, are associated with increased risks of non-Hodgkin lymphoma (NHL). There have been a few reports of cases with IgG4-related disease that had subsequently developed NHL, however, all of them suffered from B-cell lymphoma. We describe the first case of NHL, compatible with a subtype of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), which arose in IgG4-related sclerosing cholangitis. As patients with IgG4-related disease may be at an increased risk of developing NHL, such presentation during the follow-up of IgG4-related disease should be carefully scrutinized to exclude NHL.

The Expression of IgG and IgG4 in Lymphoma

“…Recently some researches showed that patients with IgG4-RD were at the risk of malignant tumors [10,11] or hematological malignancies such as lymphoma [12,13]. IgG4-RD can happen before or after the diagnosis of lymphoma.…”

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

“…В основном это экстранодальные лимфомы из клеток маргинальной зоны (MALT-лимфомы) [13][14][15][16], есть описание 1 фолликулярной лимфомы [14], 1 фолликулярной лимфомы in situ [17], 1 лимфо-мы из малых лимфоцитов [18], несколько случаев В-крупно-клеточной лимфомы [19][20][21] и одна Т-клеточная лимфома [22]. В литературе имеется описание сочетания лимфомы Ходжкина и НХЛ [23].…”

“…В литературе описано 9 случаев НХЛ, в которых диагноз достоверного IgG4-СЗ предшествовал развитию ЛПЗ или уста-навливался одновременно с диагностикой лимфомы [14,16,19,21,22].…”

“…Многие пациенты имели лимфаденопатию. Боль-шинство представленных в мировой литературе лимфом отно-сятся к В-клеточным [15-21, 23, 27], описан лишь один случай Т-клеточной лимфомы [22]. В 3 описаниях имелось сочетание различных лимфом: 2 разных MALT-лимфом одновременно [15,27] и MALT-лимфомы и лимфомы Ходжкина [23].…”

“…[22]. Кроме того, при обследовании у пациента выявлена еще солидная опухоль -высокодифферен-цированная аденокарцинома поперечной ободочной кишки.…”