Atypical Phenotype and Treatment Response Pattern in a Patient with FIP1L1-PDGFR α Mutation

We present the case of a 71-year-old male who presented with abdominal discomfort, fevers, and leukocyt osis with eosinophilia. The patient was diagnosed with a myeloproliferative neoplasm with eosinophilia and FIP1L1-PDGFRα rearrangement after a bone marrow evaluation revealed hypercellular marrow with eosinophilia and fluorescence in situ hybridization identified the FIP1L1-PDGFRα rearrangement. T he patient was successfully treated with imatinib. Within months he relapsed and converted into acute myeloid leukemia. The patient was then treated with ponatinib which induced and maintained clinical and hematological remission for 2 months. That ponatinib briefly induced remission in our patient with acute myeloid leukemia arising from a myeloproliferative neoplasm with eosinophilia and FIP1L1-PDGFRα fusion may merit exploration of ponatinib as a potential second-line treatment option for this patient population. This is especially true given the lack of reliable therapies in instances of imatinib resistance.Acta Haematol 2018;140:67 –70
Source: Acta Haematologica - Category: Hematology Source Type: research