Pulmonary Polymorphic Centroblastic Type Malignant Lymphoma in a Patient with Lymphomatoid Granulomatosis, Sjögren Syndrome and Other Manifestations of a Dysimmune State

Lymphomatoid Granulomatosis: A Unique Complication of Crohn Disease and Its Treatment in Pediatrics

“…The understanding of the pathogenesis has evolved with the identification of EBV in most cases of LYG (16,17). LYG is linked with immunosuppressive conditions, both congenital and acquired (4)(5)(6)(7)(8)(9)(10)(11). Thus, an impaired T-cell immunity may be responsible for the uncontrolled proliferation of EBV-infected B lymphocytes.…”

“…Three different grades of the disease are described, the most severe grade (grade III) being a large B-cell lymphoma (3). Patients with a primary or secondary immunodeficiency are at increased risk for LYG (2,(4)(5)(6)(7)(8)(9)(10)(11).We report a case of LYG in a patient with Crohn disease (CD). This description also identifies an EBV-driven B-cell lymphoma in a pediatric patient following 6-mercaptopurine (6-MP) immunosuppression for inflammatory bowel disease (IBD).…”

“…Three different grades of the disease are described, the most severe grade (grade III) being a large B-cell lymphoma (3). Patients with a primary or secondary immunodeficiency are at increased risk for LYG (2,(4)(5)(6)(7)(8)(9)(10)(11).…”

Lymphadenopathy, cough, and fever in a 51-year-old woman with systemic lupus erythematosus: case report of lymphomatoid granulomatosis

“…Lymphomatoid granulomatosis is a rare, often fatal lymphoproliferative disease that is most common in immunosuppressed patients such as those with acquired immunodeficiency syndrome, organ transplant, or Wiskott-Aldrich syndrome. It has been described in patients with a variety of autoimmune disorders including Sjö gren's syndrome (1), ulcerative colitis, rheumatoid arthritis (2), Crohn's disease, Hashimoto thyroiditis, and juvenile rheumatoid arthritis (3). This report is the first to describe lymphomatoid granulomatosis in a patient with systemic lupus erythematosus (SLE).…”

Treatment of Lymphomatoid Granulomatosis: Old and New Perspectives

“…The focus of research and controversy in recent years has been to characterize more precisely the nature of the malignant process. Although some cases have been identified as B-cell neoplasms, [9][10][11] most studies have suggested a T-cell origin, [12][13][14][15][16] and many recent cases of lymphomatoid granulomatosis have been reported as peripheral T-cell lymphoma or angiocentric T-cell lymphoma. 17,18…”