HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Platelet-derived growth factor receptor-alpha-associated hypereosinophilic syndrome and lymphomatoid papulosis.

Abstract
Fip1-like 1/platelet-derived growth factor receptor-alpha (FIP1L1/PDGFRA)-positive hypereosinophilic syndrome is a rare disorder with a poor prognosis if untreated and for which treatment with imatinib mesilate is highly effective. A 33-year-old man presented with recurrent papular skin lesions and marked peripheral eosinophilia. Skin biopsy revealed proliferation of CD30(+) T cells consistent with lymphomatoid papulosis (LyP), whereas molecular analysis of peripheral blood mononuclear cells demonstrated the presence of the FIP1L1/PDGFRA fusion gene. As the presence of this gene has important prognostic and therapeutic implications, this report underscores the importance of molecular testing in the evaluation of patients with LyP and peripheral eosinophilia.
AuthorsT McPherson, E W Cowen, E McBurney, A D Klion
JournalThe British journal of dermatology (Br J Dermatol) Vol. 155 Issue 4 Pg. 824-6 (Oct 2006) ISSN: 0007-0963 [Print] England
PMID16965435 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Benzamides
  • Oncogene Proteins, Fusion
  • Piperazines
  • Protein Kinase Inhibitors
  • Pyrimidines
  • mRNA Cleavage and Polyadenylation Factors
  • Imatinib Mesylate
  • FIP1L1-PDGFRA fusion protein, human
  • Receptor, Platelet-Derived Growth Factor alpha
Topics
  • Adult
  • Benzamides
  • Humans
  • Hypereosinophilic Syndrome (diagnosis, drug therapy, genetics)
  • Imatinib Mesylate
  • Lymphomatoid Papulosis (diagnosis, drug therapy, genetics)
  • Male
  • Oncogene Proteins, Fusion (genetics)
  • Piperazines (therapeutic use)
  • Protein Kinase Inhibitors (therapeutic use)
  • Pyrimidines (therapeutic use)
  • Receptor, Platelet-Derived Growth Factor alpha (genetics)
  • mRNA Cleavage and Polyadenylation Factors (genetics)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: