Imatinib treatment of lymphomatoid papulosis associated with myeloproliferative hypereosinophilic syndrome presenting the FIP1L1-PDGFRA fusion gene.

Abstract

Peripheral blood eosinophilia has been reported to occur in a wide range of haematological malignancies including primary cutaneous lymphomas. The concurrence of lymphomatoid papulosis (LyP) with peripheral blood eosinophilia seems to be an uncommon phenomenon. Atypical lymphocytes in LyP display the phenotype of activated T-helper cells, consistently express CD30 antigen, have a Th2 cytokine profile, and secrete eosinophilstimulating cytokines. Cases of LyP have been reported in association with myeloproliferative hypereosinophilic syndrome (M-HES) presenting the Fip1-like 1/plateletderived growth factor receptor-α (FIP1L1-PDGFRA) fusion gene. In such cases, imatinib treatment may lead to a complete and persistent resolution of LyP lesions.