We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Myeloid/Lymphoid Neoplasms with Eosinophilia and TKI Fusion Genes: Treatment.
- Authors
Reiter, Andreas; Cross, Nicholas C.P.; Gotlib, Jason
- Abstract
The WHO 2017 classification contains a distinct subcategory within the myeloid neoplasms as 'Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2' (MLN-eo).1 The clinical characteristics include features of myeloproliferation, usually (but not invariably) with eosinophilia, and a variable incidence of myeloid or lymphoid blast phase (PB)/acute leukemia (including extramedullary sites) at diagnosis or during disease transformation.1–4 Conventional karyotyping and/or FISH analysis reveal reciprocal translocations that create fusions between various partner genes and the tyrosine kinases (TK) PDGFRA (4q12), PDGFRB (5q31-33), FGFR1 (8p11), and JAK2 (9p24), leading to constitutive activation of the TK. More than 75 distinct TK fusion genes have been described, including some that are cytogenetically cryptic due to deletions, inversions, insertions, or rarely, complex translocations. FIP1L1-PDGFRA, which needs to be identified by routine screening through RT-PCR or FISH, is prototypic of this disease group and is the most frequent TK fusion within the subcategory of MLN-eo.2–4 In recent years, several different cytogenetically cryptic TK fusion genes have been identified through RNA sequencing, for which PDGFRB is the most frequently involved TK.5–8
- Publication
Clinical Lymphoma, Myeloma & Leukemia, 2021, Vol 21, pS66
- ISSN
2152-2650
- Publication type
Article
- DOI
10.1016/S2152-2650(21)01213-1