Two Cases With Features of Lymphocyte Variant Hypereosinophilic Syndrome With STAT3 SH2 Domain Mutations.

Fernandez-Pol, Sebastian; Petersen, Bruce; Murphy, Jo-Ellen; Oak, Jean S; Wang, Erica B K; Rieger, Kerri E; Kim, Youn H; Khodadoust, Michael S et al. · Am J Surg Pathol · 2021

case_report · Level V

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Abstract

Lymphocyte variant hypereosinophilic syndrome (LV-HES) is a rare cause of eosinophilia that is due to eosinophilipoietic cytokine production by an immunophenotypically abnormal T-cell clone. The molecular pathogenesis of this disorder is largely unknown and only 1 case of LV-HES with a pathogenic STAT3 mutation has been described thus far. Here we report 2 cases of LV-HES with STAT3 SH2 domain mutations. These cases further support the model that activation of STAT3 signaling through STAT3 SH2 domain mutations is a recurrent event in LV-HES.

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