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
Review

Myeloid/Lymphoid Neoplasms with Eosinophilia and Platelet Derived Growth Factor Receptor Alpha (PDGFRA) Rearrangement

Prabhjot Kaur  1 , Wahab A. Khan  1
Weijie Li  1
, editors.
In: Leukemia [Internet]. Brisbane (AU): Exon Publications; 2022 Oct 16. Chapter 8.
Affiliations
Review

Myeloid/Lymphoid Neoplasms with Eosinophilia and Platelet Derived Growth Factor Receptor Alpha (PDGFRA) Rearrangement

Prabhjot Kaur et al.

Excerpt

Myeloid and lymphoid neoplasms with eosinophilia and PDGFRA rearrangements are recognized as a distinct entity within the section of “Myeloid/Lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions (MLN-TK)”. Based on the WHO classification of tumors of Hematopoietic and Lymphoid tissues (5th Ed, 2022), these neoplasms also comprise of PDGFRB rearrangement, FGFR1 gene rearrangements, JAK2 rearrangement, FLT3 rearrangement, ETV6::ABL1 fusion as well as some others. Of note, these are recently defined entities and are still evolving. In this chapter, practical issues regarding diagnosis, cytogenetic testing, molecular studies, therapeutics, and clinical implications are discussed. Differential diagnosis in consideration for eosinophilia and hypereosinophilic syndrome are further highlighted.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest: The authors declare no potential conflict of interest with respect to research, authorship and/or publication of this chapter.

References

    1. Swerdlow SH, International Agency for Research on Cancer. World Health Organization . WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon, France: International Agency for Research on Cancer; 2008. p. 439.
    1. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90. https://doi.org/10.1182/blood-2016-01-643569 . - DOI - PMC - PubMed
    1. Khoury JD, Solary E, Abla O, Akkari Y, Alaggio R, Apperley JF, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms. Leukemia. 2022;36(7):1703–19. https://doi.org/10.1038/s41375-022-01613-1 . - DOI - PMC - PubMed
    1. Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. The New England journal of medicine. 2003;348(13):1201–14. https://doi.org/10.1056/NEJMoa025217 . - DOI - PubMed
    1. Metzgeroth G, Walz C, Score J, Siebert R, Schnittger S, Haferlach C, et al. Recurrent finding of the FIP1L1-PDGFRA fusion gene in eosinophilia-associated acute myeloid leukemia and lymphoblastic T-cell lymphoma. Leukemia. 2007;21(6):1183–8. https://doi.org/10.1038/sj.leu.2404662 . - DOI - PubMed

LinkOut - more resources