Watershed infarction in hypereosinophiuc syndrome: A diagnostic dilemma in FIP1L1-PDGFR alpha-associated myeloid neoplasm

Imelda Marton, Éva Pósfai, János Kristóf Annus, Zita Borbényi, Attila Nemes, L. Vécsei, E. Vörös

Research output: Contribution to journalArticle

Abstract

Introduction - The FIP1L1 -PDGFR alpha-positive, hypere-osinophilic syndrome (HES) is a new category of hematological entities. Various clinical symptoms may occur, with no specific characteristics in either the clinical picture or the neuroimaging findings, and this may give rise to a diagnostic dilemma. A report on a long follow-up period (10 years) in a case of HES that presented with neuropsychiatric symptoms appears to be unique. Besides the complexity of the diagnostic process, the successful treatment is discussed. Case report - The HES was diagnosed in a male patient at the age of 33 years, with involvement of the central nervous system and the myocardium. After the onset of the clinical signs, the MRI indicated bilateral cerebral and cerebellar cortico-subcortical lesions involving the watershed areas, mainly in the parieto-occipital regions. High-dose intravenous steroid (methylprednisolone 500 mg/day) alleviated the neurological symptoms within a few weeks, and the administration of imatinib (200 mg/day) resulted in an impressive regression of the hypereosinophilia and splenomegaly within 6 weeks. During the follow-up, the patient has continued to receive imatinib. The molecular remission has persisted, no new complaints have developed and the condition of the patient has remained stable. Conclusion - The timely recognition of the HES and identification of the disease subtype which led to the administration of imatinib may be the key to successful treatment. The long stable follow-up period gives rise to a new dilemma in the treatment of the HES in these special cases: for how long should a patient receive a tyrosine kinase inhibitor, and may the treatment be suspended?

Original languageEnglish
Pages (from-to)212-216
Number of pages5
JournalIdeggyógyászati szemle
Volume68
Issue number5-6
DOIs
Publication statusPublished - May 30 2015

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Platelet-Derived Growth Factor alpha Receptor
Infarction
Neoplasms
Occipital Lobe
Splenomegaly
Methylprednisolone
Therapeutics
Neuroimaging
Protein-Tyrosine Kinases
Myocardium
Central Nervous System
Steroids
Imatinib Mesylate

Keywords

  • CNS involvement
  • Hypereosinophilia
  • Stroke

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Watershed infarction in hypereosinophiuc syndrome : A diagnostic dilemma in FIP1L1-PDGFR alpha-associated myeloid neoplasm. / Marton, Imelda; Pósfai, Éva; Annus, János Kristóf; Borbényi, Zita; Nemes, Attila; Vécsei, L.; Vörös, E.

In: Ideggyógyászati szemle, Vol. 68, No. 5-6, 30.05.2015, p. 212-216.

Research output: Contribution to journalArticle

Marton, Imelda ; Pósfai, Éva ; Annus, János Kristóf ; Borbényi, Zita ; Nemes, Attila ; Vécsei, L. ; Vörös, E. / Watershed infarction in hypereosinophiuc syndrome : A diagnostic dilemma in FIP1L1-PDGFR alpha-associated myeloid neoplasm. In: Ideggyógyászati szemle. 2015 ; Vol. 68, No. 5-6. pp. 212-216.
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AU - Annus, János Kristóf

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AU - Nemes, Attila

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AB - Introduction - The FIP1L1 -PDGFR alpha-positive, hypere-osinophilic syndrome (HES) is a new category of hematological entities. Various clinical symptoms may occur, with no specific characteristics in either the clinical picture or the neuroimaging findings, and this may give rise to a diagnostic dilemma. A report on a long follow-up period (10 years) in a case of HES that presented with neuropsychiatric symptoms appears to be unique. Besides the complexity of the diagnostic process, the successful treatment is discussed. Case report - The HES was diagnosed in a male patient at the age of 33 years, with involvement of the central nervous system and the myocardium. After the onset of the clinical signs, the MRI indicated bilateral cerebral and cerebellar cortico-subcortical lesions involving the watershed areas, mainly in the parieto-occipital regions. High-dose intravenous steroid (methylprednisolone 500 mg/day) alleviated the neurological symptoms within a few weeks, and the administration of imatinib (200 mg/day) resulted in an impressive regression of the hypereosinophilia and splenomegaly within 6 weeks. During the follow-up, the patient has continued to receive imatinib. The molecular remission has persisted, no new complaints have developed and the condition of the patient has remained stable. Conclusion - The timely recognition of the HES and identification of the disease subtype which led to the administration of imatinib may be the key to successful treatment. The long stable follow-up period gives rise to a new dilemma in the treatment of the HES in these special cases: for how long should a patient receive a tyrosine kinase inhibitor, and may the treatment be suspended?

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