Skewed lymphocyte subpopulations and associated phenotypes in patients with mastocytosis

2019 
Abstract Background Mastocytosis is a clonal mast cell disorder associated with elevated mast cell mediators which themselves have been reported to affect lymphocyte function. However, the impact of an expanded mast cell compartment upon lymphocyte subpopulations, and their correlation with clinical phenotypes in patients with indolent systemic mastocytosis (ISM) has not been explored. Objective The goal of this prospective study is to examine the immunophenotype of circulating lymphocytes in patients with ISM compared to healthy adult controls and examine relationships with aspects of clinical disease. Methods We examined lymphocyte subsets in 20 adult patients with ISM and 40 healthy adult volunteers by multiparameter flow cytometry. Results were correlated with clinical characteristics. Results Patients with ISM exhibited a significantly lower median frequency and absolute cell count of both circulating CD8 + T cells and Natural Killer cells accompanying a significantly increased ratio of CD4 + /CD8 + T cells when compared to healthy volunteers. Stratification of our ISM patient cohort according to clinical manifestations revealed that CD19 + CD21 low CD38 low B cells were significantly higher in patients with a history of autoimmune disease and counts of terminally differentiated CD4 + T cells were significantly higher in patients with osteoporosis or osteopenia. Conclusions Several circulating lymphocyte subpopulations in patients with ISM were significantly different when compared to healthy controls; and in specific lymphocyte subsets, this lymphocyte skewing correlated with clinical observations including osteoporosis and autoimmune disease. These data suggest the need for further studies on abnormalities in lymphocyte subsets and the attendant clinical consequences in both mast cell proliferative and activation disorders.
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