Metachronous development of Hodgkin’s lymphoma and nodal marginal zone lymphoma in a patient with Sjögren’s syndrome: a case report

According to various population-based studies, lymphoproliferative disorders develop 2–10 times more frequently in individuals with autoimmune pathology compared to the general population. Patients with Sjögren's disease/syndrome exhibit 10–44 times increased risk of lymphoma development compared to the general population. This predisposition is primarily driven by chronic B-cell stimulation from autoantigens produced by exocrine glands and other organs. However, no clear therapeutic guidelines for the management of these patients have been published.

This report presents a unique case of metachronous development of Hodgkin's lymphoma and nodal marginal zone lymphoma in a patient with Sjögren’s syndrome. The diagnostic workup is described in detail, including histopathological and immunohistochemical findings as well as imaging studies, along with the therapeutic approaches employed. The main difficulty in treatment was the alternation of recurrent course of lymphomas amid immunological dysregulation and autoaggression aggravated by checkpoint inhibitors, especially immune damage to the liver. The issue of the formation of an optimal therapeutic approach in this category of patients is raised in this article. The article examines the relationship between autoimmune pathology and lymphoproliferative disorders, addressing fundamental questions about the primacy of pathological processes and the development of optimal diagnostic and therapeutic approaches for this patient category.