Abstract
Objective: Eosinophilia associated with FIP1L1-PDGFRA rearrangement represents a subset of chronic eosinophilic leukemia and affected patients are sensitive to imatinib treatment. This study was undertaken to learn the prevalence and associated clinicopathologic and genetic features of FIP1L1-PDGFRA rearrangement in a cohort of 26 adult patients presenting with profound eosinophilia (>1.5x109/L).Materials and Methods: Reverse-transcriptase polymerase chain reaction and gel electrophoresis were used for the detection of FIP1L1-PDGFRA rearrangement. Results: Five male patients with splenomegaly carried the FIP1L1-PDGFRA gene rearrangement. All patients achieved complete hematological response within 4 weeks of starting imatinib. One patient had previous deep vein thrombosis and 1 patient had cardiomyopathy, which improved with steroids and imatinib. Conventional cytogenetics was normal in all these patients. No primary resistance to imatinib was noted.Conclusion: This study indicates the need to do the FIP1L1-PDGFRA assay in patients with hypereosinophilic syndrome. Prompt treatment of this condition with imatinib can lead to complete hematological response and resolution of the organ damage that can be seen in this setting.
Highlights
Hypereosinophilic syndrome (HES) is an uncommon disorder with persistent eosinophilia and multiple organ dysfunction due to eosinophilic infiltration [1]
Clonal or neoplastic eosinophilia is defined as eosinophilia originating from the malignant clone in hematopoietic stem cells and myeloid neoplasms
All patients were started on imatinib at 100 mg or 400 mg per day as per the physician’s preference
Summary
Hypereosinophilic syndrome (HES) is an uncommon disorder with persistent eosinophilia and multiple organ dysfunction due to eosinophilic infiltration [1]. The spectrum of clinical manifestations are variable and patients may be asymptomatic or may have endomyocardial fibrosis or restrictive lung disease. Myeloproliferative neoplasm with eosinophilia and plateletderived growth factor receptor-alpha gene and Fip1-like 1 gene mutation (FIP1L1-PDGFRA; F/P) is a low-burden disease with dramatic response to imatinib therapy. Various classifications over the last 20 years, and especially from the Year 2011 Working Conference on Eosinophil Disorders and Syndromes, have tried to give us a better understanding of the pathophysiology and management of this rare but clinically important and treatable hematological malignancy [1]. We undertook this study to learn the clinical profile and outcome in our region
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Schedule a callMore From: Turkish journal of haematology : official journal of Turkish Society of Haematology
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