[FIP1L1::PDGFRA-positive chronic eosinophilic leukemia showing slowly progressive cardiac impairment]

Rinsho Ketsueki. 2022;63(12):1648-1652. doi: 10.11406/rinketsu.63.1648.
[Article in Japanese]

Abstract

This report describes a 69-year-old man with eosinophilia that was detected during a medical check-up. A review of his medical history revealed that his absolute eosinophil count was 990/µl at the age of 55 and increased to 5,380/µl at the age of 69. Electrocardiogram revealed first-degree atrioventricular block at the age of 58, followed by ST-segment depression and a negative T wave at the age of 65. The echocardiogram was normal at the age of 65. We diagnosed him with FIP1L1::PDGFRA-positive chronic eosinophilic leukemia by detecting the FIP1L1::PDGFRA fusion gene by fluorescence in situ hybridization. He had no symptoms at the first visit; however, echocardiography and contrast-enhanced computed tomography revealed an abnormal structure, considered a thrombus, within the left ventricular apex and apex wall thickening. We initiated imatinib (100 mg/day), and the eosinophilia disappeared in a month. However, his cardiac impairment worsened, and he gradually developed symptoms of heart failure. This case is valuable because it shows the long-term course of the disease, with 15 years of laboratory findings until diagnosis. Our findings suggest that in cases of eosinophilia with an abnormal electrocardiogram, contrast-enhanced cardiac magnetic resonance imaging should be considered earlier.

Keywords: Cardiac impairment; FIP1L1::PDGFRA; Hypereosinophilic syndrome; Imatinib.

Publication types

  • Case Reports
  • Review
  • English Abstract

MeSH terms

  • Aged
  • Benzamides
  • Humans
  • Hypereosinophilic Syndrome* / drug therapy
  • In Situ Hybridization, Fluorescence
  • Male
  • Oncogene Proteins, Fusion / genetics
  • Piperazines*
  • Pyrimidines
  • Transcription Factors / genetics

Substances

  • Piperazines
  • Pyrimidines
  • Benzamides
  • Transcription Factors
  • Oncogene Proteins, Fusion

Supplementary concepts

  • Pdgfra-Associated Chronic Eosinophilic Leukemia