Abstract
Mastocytosis is a rare neoplastic disease of the bone marrow. Common symptoms like urticaria, diarrhea, bronchspasm and flushing are caused by mast cell degranulation and are mostly based on mast cell mediator release and Th2 type inflammation that occurs frequently in these patients. Psychological disorders are more prevalent in patients with systemic mastocytosis, though little is known about the mechanism behind this. The aim of the study was to investigate the Th2 cytokine (IL-4, TSLP, IL-31 and IL-33) profile in patients with mastocytosis in relation to classic degranulation symptoms and the psychometric measures of cognition and distress symptoms.In total, 115 patients diagnosed with mastocytosis were enrolled. Mini-Mental State Examination (MMSE) was performed for all subjects. Other variables: Quality of life in mastocytosis, a mood assessment commonly used in systemic mastocytosis by a certified rater-the Hamilton-17 Depression Scale, Pruritus Visual Analog Score, serum tryptase concentration and bone marrow biopsy results (archival) were also analyzed/included. Serum concentrations of IL-4, TSLP, IL-33 and IL-31 were analyzed as primary outcomes. For comparison with continuous variables linear regression was used. The mean MMSE result was 27.9. Regression analysis did not reveal significant correlation between the IL-4 (p = 0.82), IL-31 (p = 0.24) and TSLP (p = 0.37) serum concentrations and MMSE. The IL-33 concentration analysis resulted in 0 for all patients (was not detected). No significant effect was observed with other endpoints as well. One in four patients with mastocytosis presents cognitive decline. This impairment does not correlate with Il-4, TSLP, nor IL-31 serum protein concentrations.