Related Experiment Video
Updated: Jul 19, 2026
09:01
Flow Cytometry to Estimate Leukemia Stem Cells in Primary Acute Myeloid Leukemia and in Patient-derived-xenografts, at Diagnosis and Follow Up
Published on: March 26, 2018
[A man with FIP1L1/PDGFRA-positive chronic eosinophilic leukemia]
O C Leeksma1, G S de Ruiter, V P M van der Hulst
1Onze Lieve Vrouwe Gasthuis, Oosterpark, HM Amsterdam. o.c.leeksma@olvg.nl
Nederlands Tijdschrift Voor Geneeskunde
|September 27, 2006
Summary
A patient with chronic eosinophilic leukemia and a heart tumor achieved complete remission with imatinib therapy. This targeted treatment normalized blood counts and eliminated the fusion gene, demonstrating its effectiveness in managing this rare condition.
Area of Science:
- Hematology
- Oncology
- Cardiology
Background:
- Chronic eosinophilic leukemia (CEL) is a rare myeloproliferative neoplasm characterized by persistent eosinophilia.
- The FIP1L1-PDGFRA fusion gene is a key molecular driver in a subset of CEL cases, often associated with cardiac involvement.
- Right ventricular tumors, potentially linked to hypereosinophilia, present a diagnostic and therapeutic challenge.
Observation:
- A 39-year-old male presented with hypereosinophilia, mild thrombocytopenia, and a right ventricular tumor.
- Diagnostic evaluation revealed the presence of the FIP1L1-PDGFRA fusion gene, confirming CEL.
- The cardiac mass had been present for at least six months without causing significant hemodynamic compromise.
Findings:
- Treatment with oral anticoagulants and imatinib (a tyrosine kinase inhibitor) led to normalization of leukocyte counts and differential values.
- Within three months, the FIP1L1-PDGFRA fusion transcript became undetectable in peripheral blood.
- After one year, the patient achieved complete hematological and molecular remission of CEL, with the cardiac mass remaining stable.
Implications:
- Imatinib is a highly effective targeted therapy for CEL associated with the FIP1L1-PDGFRA fusion gene.
- This case highlights the successful management of CEL with cardiac involvement using tyrosine kinase inhibitors.
- Long-term monitoring is crucial for patients with CEL and cardiac masses, even after achieving remission.
