title: Unique association of myeloid neoplasm with eosinophilia and abnormalities of PDGFRA with TTP.
abstract: Myeloid neoplasm with eosinophilia and abnormalities of Alpha type platelet derived growth factor receptor (PDGFRA) is a type of hypereosinophilic syndrome characterized by multiorgan damage due to eosinophilia. Its association with thrombotic thrombocytopenic purpura (TTP) has rarely been reported. We describe here a case report of a female in whom TTP presented as one of the earlier manifestations of myeloproliferative HES with rearrangement of PDGFRA. Our patient was found to have a normal ADAMTS-13 level which is not commonly seen with TTP. This case illustrates the importance of recognizing the atypical presentations of HES which may be difficult to recognize.
authors.affiliation: Dept. of Internal Medicine, West Virginia University, Morgantown, WV 26506, USA. lchaudhary@hsc.wvu.edu
authors.forename: Lubna N
authors.initials: LN
authors.lastname: Chaudhary
authors.forename: Nathanael G
authors.initials: NG
authors.lastname: Bailey
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Vos
authors.forename: Christy J
authors.initials: CJ
authors.lastname: Stotler
mesh_terms: D000328:Adult; D005260:Female; D015321:Gene Rearrangement; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000175:diagnosis* / Q000235:genetics; D009196:Myeloproliferative Disorders / Q000175:diagnosis* / Q000235:genetics; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics*; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics*
chemical_list: D015514:Oncogene Proteins, Fusion; D039221:mRNA Cleavage and Polyadenylation Factors; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha