Myeloid/lymphoid neoplasms with FGFR1 rearrangement

Myeloid/lymphoid neoplasms with FGFR1 rearrangement are a rare entity. We present a multicenter experience of 17 patients with FISH-confirmed FGFR1 rearrangement. The clinical presentation at diagnosis included myeloproliferative neoplasm (MPN) in 4 (24%) patients, acute leukemia (AL) in 7 (41%), an...

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Bibliographic Details
Published in:Leukemia & lymphoma 2018-07, Vol.59 (7), p.1672-1676
Main Authors: Strati, Paolo, Tang, Guilin, Duose, Dzifa Y., Mallampati, Saradhi, Luthra, Rajyalakshmi, Patel, Keyur P., Hussaini, Mohammad, Mirza, Abu-Sayeef, Komrokji, Rami S., Oh, Stephen, Mascarenhas, John, Najfeld, Vesna, Subbiah, Vivek, Kantarjian, Hagop, Garcia-Manero, Guillermo, Verstovsek, Srdan, Daver, Naval
Format: Article
Language:English
Subjects:
acute leukemia
Adolescent
Adult
Aged
Biomarkers
Biomarkers, Tumor
Child
Child, Preschool
DNA Mutational Analysis
Female
FGFR1 rearrangement
Gene Rearrangement
Genetic Association Studies
Genetic Predisposition to Disease
Humans
In Situ Hybridization, Fluorescence
Infant
Karyotype
Leukemia, Lymphoid - diagnosis
Leukemia, Lymphoid - genetics
Leukemia, Lymphoid - mortality
Leukemia, Lymphoid - therapy
Lymphoma - diagnosis
Lymphoma - genetics
Lymphoma - mortality
Lymphoma - therapy
Male
Middle Aged
Myeloproliferative Disorders - diagnosis
Myeloproliferative Disorders - genetics
Myeloproliferative Disorders - mortality
Myeloproliferative Disorders - therapy
Myeloproliferative neoplasm
Prognosis
Receptor, Fibroblast Growth Factor, Type 1 - genetics
RUNX1
Translocation, Genetic
Young Adult
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Summary:Myeloid/lymphoid neoplasms with FGFR1 rearrangement are a rare entity. We present a multicenter experience of 17 patients with FISH-confirmed FGFR1 rearrangement. The clinical presentation at diagnosis included myeloproliferative neoplasm (MPN) in 4 (24%) patients, acute leukemia (AL) in 7 (41%), and concomitant MPN with AL in 6 (35%). The two most frequently observed cytogenetic abnormalities were t(8;13)(p11.2;q12)(partner gene ZMYM2) and t(8;22)(p11.2; q11.2)(BCR). Seventy-eight percent of tested patients had a RUNX1 mutation, of whom all had AL. Overall response rate to frontline therapy was 69%, and 76% of patients subsequently received allogeneic stem cell transplant (ASCT). After a median follow-up of 11 months, median progression-free survival was 15 months and median overall survival was not reached. In conclusion, FGFR1-rearranged hematologic malignancies present with features of MPN and/or AL. FGFR1 and RUNX1 are therapeutic targets for ongoing and future clinical trials.
ISSN:1042-8194
1029-2403
DOI:10.1080/10428194.2017.1397663