FIP1L1–PDGFRA positive chronic eosinophilic leukaemia and associated central nervous system involvement
Interstitial deletion involving chromosome 4q12 generates the novel tyrosine kinase fusion protein encoded by FIP1L1–PDGFRA, which is present in many patients previously labelled as having hypereosinophilic syndrome, initially reported in 2003. Reports in recent literature document excellent clinica...
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Published in | Journal of clinical pathology Vol. 61; no. 5; pp. 677 - 680 |
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Main Authors | , , , , , , |
Format | Journal Article |
Language | English |
Published |
England
BMJ Publishing Group Ltd and Association of Clinical Pathologists
01.05.2008
BMJ Publishing Group LTD |
Subjects | |
Online Access | Get full text |
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Summary: | Interstitial deletion involving chromosome 4q12 generates the novel tyrosine kinase fusion protein encoded by FIP1L1–PDGFRA, which is present in many patients previously labelled as having hypereosinophilic syndrome, initially reported in 2003. Reports in recent literature document excellent clinical and molecular response to the tyrosine kinase inhibitor imatinib (Glivec). This report describes the case of a 58-year-old lady, diagnosed with FIP1L1–PDGFRA positive hypereosinophilic disorder, who subsequently developed symptoms related to an intracranial lesion. Biopsy and molecular genetic studies confirmed a diffuse infiltrative lesion, with evidence of FIP1L1–PDGFRA gene fusion. Initiation of imatinib treatment led to impressive clinical and radiological response. |
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Bibliography: | href:jclinpath-61-677.pdf ArticleID:cp52100 PMID:18256119 istex:7AC6F98179F293AAD072645426F63757EA39A28E ark:/67375/NVC-5NL2MK03-2 local:jclinpath;61/5/677 ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Case Study-2 ObjectType-Feature-4 ObjectType-Report-1 ObjectType-Article-3 |
ISSN: | 0021-9746 1472-4146 |
DOI: | 10.1136/jcp.2007.052100 |