Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017 Apr;22(4):480-483.
doi: 10.1634/theoncologist.2016-0354. Epub 2017 Feb 27.

Response to Tyrosine Kinase Inhibitors in Myeloproliferative Neoplasia with 8p11 Translocation and CEP110- FGFR1 Rearrangement

Marc Wehrli  1 , Elisabeth Oppliger Leibundgut  2 , Heinrich H Gattiker  3 , Markus G Manz  1 , Antonia M S Müller  4 , Jeroen S Goede  1
Affiliations
Case Reports

Response to Tyrosine Kinase Inhibitors in Myeloproliferative Neoplasia with 8p11 Translocation and CEP110- FGFR1 Rearrangement

Marc Wehrli et al. Oncologist. 2017 Apr.

Abstract

This brief communication reports on a patient with an exceedingly rare "8p11 (eight-p-eleven) myeloproliferative syndrome" (EMS) with CEP110-FGFR1 rearrangement who responded to treatment with the multi-tyrosine kinase inhibitor (TKI) dasatinib. Dasatinib improved quality of life substantially by increasing blood counts and reducing the need for transfusions. This report demonstrates that the second-generation TKI may provide a therapeutic option for elderly and frail EMS patients who cannot be offered aggressive therapy, including allogeneic hematopoietic cell transplantation. The Oncologist 2017;22:480-483.

Keywords: Acute myeloid leukemia; CEP110‐FGFR1 fusion protein; Dasatinib; Eosinophilia; Imatinib.

PubMed Disclaimer

Conflict of interest statement

Disclosures of potential conflicts of interest may be found at the end of this article.

Figures

Figure 1.
Figure 1.
Dynamics of the disease as assessed by CEP110‐FGFR1, hemogram, and number of transfusions. (A): Gel electrophoresis of the CEP110‐FGFR1 polymerase chain reaction products (212 bp). Lane M, 100 bp ladder as a size marker; lane 1, peripheral blood (PB) at diagnosis of 8p11 myeloproliferative syndrome; lane 2, bone marrow (BM) at diagnosis of AML; lanes 3 and 4, PB and BM at relapse, respectively; lane 5, positive control. (B): Hemoglobin, reticulocyte, platelet, and eosinophil counts before, during, and after treatment with imatinib and dasatinib. Numbers of pRBC transfusions per month are displayed in the bottom panel. The solid line represents relapse of disease. Day 0 (dotted line) indicates start with imatinib. Day 63 (dotted line) indicates start of dasatinib treatment. Abbreviations: pRBC, packed red blood cell.
See this image and copyright information in PMC

References

    1. Arber DA, Orazi A, Hasserjian R et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127:2391–2405. - PubMed
    1. Yamamoto S, Otsu M, Matsuzaka E et al. Screening of drugs to treat 8p11 myeloproliferative syndrome using patient‐derived induced pluripotent stem cells with fusion gene CEP110‐FGFR1. PLoS One 2015;10:e0120841. - PMC - PubMed
    1. Guasch G, Mack GJ, Popovici C et al. FGFR1 is fused to the centrosome‐associated protein CEP110 in the 8p12 stem cell myeloproliferative disorder with t(8;9)(p12;q33). Blood 2000;95:1788–1796. - PubMed
    1. Mozziconacci MJ, Carbuccia N, Prebet T et al. Common features of myeloproliferative disorders with t(8;9)(p12;q33) and CEP110‐FGFR1 fusion: Report of a new case and review of the literature. Leuk Res 2008;32:1304–1308. - PubMed
    1. Jackson CC, Medeiros LJ, Miranda RN. 8p11 myeloproliferative syndrome: A review. Hum Pathol 2010;41:461–476. - PubMed

Publication types

MeSH terms

Supplementary concepts