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So far, it remains unknown whether expression of these antigens on MC is of prognostic significance in SM. We performed a retrospective multi-center study of patients with SM using the data set of the registry of the European Competence Network on Mastocytosis, including patients with various MC disorders.
In most patients, the disease is driven by an activating somatic point mutation in the KIT gene leading to a constitutive activation of the KIT receptor [ 1 , 2 , 3 , 4 ]. Since , the diagnosis of SM is based on histology and morphology as well as molecular and phenotypic criteria [ 1 , 2 , 3 , 4 ]. There are four minor diagnostic SM criteria.
In the presence of hereditary alpha tryptasemia, the tryptase level should be divided by the total copy numbers of the alpha tryptase gene [ 4 , 10 ].
In the context of ISM, CD2 seems to be a recurrent aberrant and functionally relevant marker of neoplastic MC [ 1 , 2 , 3 , 4 , 16 , 17 ]. However, so far, little is known about the prognostic impact of aberrantly expressed cell surface antigens on MC in SM.