Identification of Clonal Proliferation of T Cell and FIP1L1-PDGFRα Fusion Gene in Hypereosinophilic Syndrome Associated with Lymphomatoid Papulosis which Showed Rapid and Complete Response to the Treatment with Imatinib

Imatinib Treatment of Lymphomatoid Papulosis Associated with Myeloproliferative Hypereosinophilic Syndrome Presenting the FIP1L1-PDGFRA Fusion Gene

“…In 4 of these, the FIP1L1-PDGFRA fusion gene was demonstrated. A rapid regression of the cutaneous lesions was noted and a persistent, complete clinical, haematological and molecular remission was achieved (4,6). No recurrences of LyP have been observed after variable follow-up periods.…”

“…Since the original description, 11 cases of LyP associated with idiopathic HES have been reported (2)(3)(4)(5)(6)(7)(8). The associated HES corresponded to M-HES and specifically to patients presenting the FIP1L1-PDGFRA fusion gene, the most well-characterized molecular defect leading to hypereosinophilia (9).…”