Yonsei Med J.  2001 Apr;42(2):258-263. 10.3349/ymj.2001.42.2.258.

Non-Hodgkin's lymphoma & primary biliary cirrhosis with Sjogren's syndrome

• Hahn JS
• Kim C
• Min YH
• Ko YW
• Suh CO
• Park YY

• KMID: 720618
• DOI: http://doi.org/10.3349/ymj.2001.42.2.258

Abstract

Sjogren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of the salivary and lacrimal glands leading to a progressive destruction of these glands due to the production of autoantibodies. This disorder is either isolated (primary SS) or associated with other systemic diseases (secondary SS). The occurrence of B-cell non-Hodgkin's lymphoma (NHL) represents the major complication in the evolution of SS patients. The risk of developing NHL, which is equivalent for both primary and secondary SS, was estimated to be 44 times greater than that observed in a comparable normal population. NHLs in SS patients occur preferentially in the salivary glands and in other mucosa-associated lymphoid tissues (MALT). However, it can also occur in the lymph nodes or bone marrow. We documented a case of low-grade B-cell lymphoma of MALT in the right eyelid and primary biliary cirrhosis (PBC) of a patient with SS. To the best of our knowledge, this is the first case reported in Korea.

Keyword

Sjogren's syndrome; non-Hodgkin's lymphoma; PBC; eyelid

MeSH Terms

Case Report
Eyelid Neoplasms/pathology
Eyelid Neoplasms/etiology*
Female
Human
Liver Cirrhosis, Biliary/pathology
Liver Cirrhosis, Biliary/complications*
Lymphoma, Mucosa-Associated Lymphoid Tissue/pathology
Lymphoma, Mucosa-Associated Lymphoid Tissue/etiology*
Middle Age
Sjogren's Syndrome/pathology
Sjogren's Syndrome/complications*