当前位置： SCI文献检索 > EUROPEAN JOURNAL OF HAEMATOLOGY期刊下所有文献 > Synchronous FIP1L1-PDGFRA-positive chronic eosinophilic leukemia and T-cell lymphoblastic lymphoma: a bilineal clonal malignancy.

Synchronous FIP1L1-PDGFRA-positive chronic eosinophilic leukemia and T-cell lymphoblastic lymphoma: a bilineal clonal malignancy.

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.

journal_name

Eur J Haematol

journal_title

European journal of haematology

authors

Capovilla M,Cayuela JM,Bilhou-Nabera C,Gardin C,Letestu R,Baran-Marzak F,Fenaux P,Martin A

doi

10.1111/j.1600-0609.2007.00973.x

subject

Has Abstract

pub_date

2008-01-01 00:00:00

pages

81-6

issue

1

eissn

0902-4441

issn

1600-0609

pii

EJH973

journal_volume

80

pub_type

杂志文章

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