Characterization of T-cell large granular lymphocyte leukemia associated with Sjogren's syndrome-an important but under-recognized association

Seminars in Arthritis and Rheumatism
Joshua FriedmanAlain Berrebi

Abstract

Patients with T-cell (CD3+) large granular lymphocyte (LGL) leukemia have a high prevalence of autoantibodies and associated autoimmune diseases. Sjogren's syndrome may not be diagnosed unless specifically looked for. We set to determine the prevalence of Sjogren's syndrome in LGL leukemia and its cytokine profile. Every patient with a confirmed diagnosis of LGL leukemia diagnosed at a single academic medical center over the last 15 years was evaluated for Sjogren's syndrome by questioning about sicca symptoms. In symptomatic patients, Schirmer's test, rose bengal corneal staining, salivary flow rate measurement, autoantibody screening, and minor salivary gland biopsy were performed. Supernatants obtained from T-LGL leukemic cells following phytohemagglutinin (PHA) activation were analyzed for cytokine production by enzyme-linked immunosorbent assay and patients with or without Sjogren's syndrome were compared with controls. Of 48 patients, 21 reported sicca symptoms and were enrolled in the study. In 8 patients Sjogren's syndrome was ruled out. Thirteen patients had clear evidence of Sjogren's syndrome according to accepted criteria (27%). None had rheumatoid arthritis, but 1 had limited scleroderma. Thus, 12/48 patients had p...Continue Reading

References

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Aug 16, 2003·Seminars in Arthritis and Rheumatism·Paolo Manganelli, Pieranna Fietta

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Citations

Feb 12, 2008·Clinical and Experimental Dermatology·S MalloJ Santos-Juanes
Aug 1, 2009·Hematological Oncology·Ranran ZhangThomas P Loughran
Oct 28, 2014·International Journal of Rheumatic Diseases·Santoshkumar MandalDebashish Danda
Sep 25, 2012·Clinical Lymphoma, Myeloma & Leukemia·Bruno Bockorny, Constantin A Dasanu
Dec 12, 2013·Seminars in Arthritis and Rheumatism·Alexandra AudemardBoris Bienvenu
Dec 27, 2016·Clinical and Experimental Nephrology·David RibesStanislas Faguer
Feb 9, 2020·Muscle & Nerve·Vidya S LimayePravin Hissaria
Jul 11, 2020·Journal of Investigative Medicine High Impact Case Reports·Sara Naji RadPrachi Anand
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Apr 4, 2021·Cancers·Wilma BarcelliniBruno Fattizzo
Sep 18, 2021·Rheumatology·Elie NaddafTeerin Liewluck

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