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: Lubna N Chaudhary, Nathanael G Bailey, Jeffrey A Vos, Christy J Stotler
• Journal: The West Virginia medical journal (W V Med J) 2013 Mar-Apr Vol. 109 Issue 2 Pg. 6-9 ISSN: 0043-3284 [Print] United States
• PMID: 23600098 (Publication Type: Case Reports, Journal Article)

Chemical References

• Oncogene Proteins, Fusion
• mRNA Cleavage and Polyadenylation Factors
• FIP1L1-PDGFRA fusion protein, human
• Receptor, Platelet-Derived Growth Factor alpha

Topics

• Adult
• Female
• Gene Rearrangement
• Humans
• Hypereosinophilic Syndrome (diagnosis, genetics)
• Myeloproliferative Disorders (diagnosis, genetics)
• Oncogene Proteins, Fusion (genetics)
• Purpura, Thrombotic Thrombocytopenic (diagnosis)
• Receptor, Platelet-Derived Growth Factor alpha (genetics)
• mRNA Cleavage and Polyadenylation Factors (genetics)