We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Synchronous FIP1L1-PDGFRA-positive chronic eosinophilic leukemia and T-cell lymphoblastic lymphoma: a bilineal clonal malignancy.
- Authors
Capovilla, Mathieu; Cayuela, Jean-Michel; Bilhou-Nabera, Chrystèle; Gardin, Claude; Letestu, Remi; Baran-Marzak, Fanny; Fenaux, Pierre; Martin, Antoine
- Abstract
Several reports of successful empirical treatment of idiopathic hypereosinophilic syndrome with imatinib led to the recent identification of the FIP1L1-PDGFRA fusion gene rearrangement, which characterizes a distinctive group of chronic eosinophilic leukemias. This fusion gene can be detected in eosinophils, neutrophils, mast cells, T cells, B cells and monocytes in FIP1L1-PDGFRA-positive hypereosinophilic patients suggesting a multilineage involvement. Furthermore, the same FIP1L1-PDGFRA rearrangement was identified in patients with hypereosinophilia and atypical mast cell proliferations, raising the question of a disease with two concomitant lines of differentiation. In addition, a recent report noted two cases with the association of FIP1L1-PDGFRA-positive chronic eosinophilic leukemia and T-cell lymphoblastic lymphoma (T-LBL). We report here the only third case of synchronous chronic eosinophilic leukemia and T-LBL, both associated with a FIP1L1-PDGFRA fusion transcript, confirming the occurrence of such disease and suggesting a clonal proliferation with two lines of differentiation probably arising from a primitive multipotent medullary stem cell.
- Publication
European journal of haematology, 2008, Vol 80, Issue 1, p81
- ISSN
1600-0609
- Publication type
Journal Article
- DOI
10.1111/j.1600-0609.2007.00973.x