Abstract
The FIP1L1-PDGFRA rearrangement results in constitutive activation of the tyrosine kinase PDGFRA. Neoplasms harboring this rearrangement are responsive to imatinib mesylate at doses much lower than those recommended for the treatment of chronic myelogenous leukemia. Only a single report has described the identification of FIP1L1-PDGFRA in chronic myelomonocytic leukemia (CMML). Herein, we present a case report of a patient in whom the FIP1L1-PDGFRA was discovered as he evolved from CMML to acute myeloid leukemia (AML). The presence of a dominant neoplastic clone with FIP1L1-PDGFRA rearrangement was suspected on the basis of sudden onset of peripheral and bone marrow eosinophilia and confirmed by fluorescence in situ hybridization and molecular diagnostic tests. Whereas the patient was initially refractory to chemotherapy before the rearrangement was detected, subsequent therapy with imatinib led to complete remission.
Highlights
Hypereosinophilia is a feature of a variety of uncommon hematologic disorders like hyperseosinophilic syndrome (HES), systemic mastocytosis (SM) and chronic eosinophilic leukemia (CEL)
We present the case of a patient in whom FIP1L1-PDGFRA was discovered at the time of evolution from chronic myelomonocytic leukemia (CMML) to refractory acute myeloid leukemia and how therapy with imatinib resulted in durable complete remission
We describe a case report of a patient who transformed from CMML to acute myeloid leukemia (AML) which was refractory to standard chemotherapy
Summary
Hypereosinophilia is a feature of a variety of uncommon hematologic disorders like hyperseosinophilic syndrome (HES), systemic mastocytosis (SM) and chronic eosinophilic leukemia (CEL). The patient was enrolled on the SGI-110 clinical trial, a phase 1–2 dose escalation, multicenter study of SGI-110, a DNA hypomethylating agent, in subjects with intermediate or high-risk myelodysplastic syndromes (MDS) or AML He received two courses of SGI-110, after which he experienced rapidly progressive leukocytosis with a peak WBC count of 126 × 109/L and new onset of peripheral eosinophilia (11%). WBC: white blood cells, PB: peripheral blood, AEC: absolute eosinophil count, BM: bone marrow, CMML: chronic myelomonocytic leukemia, AML: acute myeloid leukemia. The patient was started on imatinib mesylate 400 mg daily along with a short course of idarubucin and subcutaneous cytarabine for cytoreduction He achieved complete hematologic and morphologic remission and went on to receive a matched unrelated allogeneic stem cell transplant (SCT).
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