Large Granular Lymphocyte Leukemia and Sjogren Syndrome: An Update of the Cases Reported in Literature and a New Clinical Perspective

Abstract

Sjogren syndrome is a chronic autoimmune disease which disproportionately affects women, characterized by intense inflammation and destruction of the exocrine glands and multi-organ involvement. Furthermore, it is commonly associated with other autoimmune and hematological disorders and the latter of which are the focus of this brief review. More specifically, the association between Sjogren syndrome and large granular lymphocyte leukemia will be explored: this hematological malignancy, characterized by a clonal expansion of either T or NK cells, is usually extremely rare, but has been reported with a certain frequency in association with autoimmune disorders. Until now, about thirty cases of an association between these two disorders have been reported: the review of their clinical and laboratory characteristics highlights frequent symptoms (cytopenia and autoimmune manifestations, especially involving the thyroid) and laboratory findings (such as an overwhelming involvement of T CD8 cells). These elements seem to underline commonalities in the pathogeneses of the two disorders, and on one side suggest that focusing on these common elements might help understand the diseases better (for example, by helping to elucidate the role of T CD8 cells in Sjogren syndrome); on the other hand, they underline how important it is to conduct a thorough and timely screening for comorbidities in cases of Sjogren syndrome or large granular lymphocyte leukemia, in order to better treat the whole spectrum of the patients’ problems.