title: WATERSHED INFARCTION IN HYPEREOSINOPHILIC SYNDROME: A DIAGNOSTIC DILEMMA IN FIP1L1-PDGFR ALPHA-ASSOCIATED MYELOID NEOPLASM.
abstract: BACKGROUND
  The FIP1L1-PDGFR alpha-positive, hypereosinophilic 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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?
authors.forename: Imelda
authors.initials: I
authors.lastname: Marton
authors.forename: Éva
authors.initials: É
authors.lastname: Pósfai
authors.forename: János Kristóf
authors.initials: JK
authors.lastname: Annus
authors.forename: Zita
authors.initials: Z
authors.lastname: Borbényi
authors.forename: Attila
authors.initials: A
authors.lastname: Nemes
authors.forename: László
authors.initials: L
authors.lastname: Vecsei
authors.forename: Erika
authors.initials: E
authors.lastname: Vörös
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics; D019046:Bone Marrow Neoplasms / Q000737:chemistry / Q000150:complications*; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis; D002493:Central Nervous System Diseases / Q000150:complications / Q000175:diagnosis; D003937:Diagnosis, Differential; D015321:Gene Rearrangement; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D000068877:Imatinib Mesylate; D007238:Infarction / Q000175:diagnosis* / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D018696:Neuroprotective Agents / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D011743:Pyrimidines / Q000627:therapeutic use; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000032:analysis* / Q000235:genetics; D039221:mRNA Cleavage and Polyadenylation Factors / Q000032:analysis* / Q000235:genetics
chemical_list: D000893:Anti-Inflammatory Agents; D000970:Antineoplastic Agents; D001549:Benzamides; D014408:Biomarkers, Tumor; C479818:FIP1L1 protein, human; D018696:Neuroprotective Agents; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases; D020796:Receptor, Platelet-Derived Growth Factor alpha; D008775:Methylprednisolone