HAEM4:Myeloid/Lymphoid Neoplasms with Eosinophilia and Rearrangement of PDGFRA, PDGFRB or FGFR1, or with PCM1-JAK2
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Primary Author(s)*
Daynna J.Wolff
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General Disease Overview / Description of Cancer Category
The World Health Organization (WHO) category, “Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2” is a group of diseases with particular gene fusions that result in dysregulated tyrosine kinase (TK) genes. This entity was originally described in the 2008 WHO classification scheme as “Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of platelet-derived growth factor receptor α (PDGFRA), platelet-derived growth factor receptor β (PDGFRB), or fibroblast growth factor receptor 1 (FGFR1).”[1] The classification was modified for the WHO 2017 to include the provisional entity of PCM1-JAK2-positive neoplasms.[2][3]
Patients with myeloid/lymphoid neoplasms with eosinophila with gene rearrangement can present clinically with myeloproliferative neoplasms (MPN), myelodysplastic syndrome/MPN, as well as de novo or secondary mixed-phenotype leukemias or lymphomas. While eosinophilia is common, it is not always a feature of these diseases.
The natural history of PDGFRA- and PDGFRB-rearranged neoplasms has been dramatically altered by imatinib. In contrast, patients with FGFR1 and JAK2 fusion TK genes exhibit a more aggressive course and variable sensitivity to current TK inhibitors, and in most cases, long-term disease-free survival may only be achievable with allogeneic hematopoietic stem cell transplantation.
WHO Classification Pages (Includes Links to Content)
- HAEM5:Myeloid/lymphoid neoplasm with PDGFRA rearrangement
- HAEM5:Myeloid/lymphoid neoplasm with PDGFRB rearrangement
- HAEM5:Myeloid/lymphoid neoplasm with FGFR1 rearrangement
- HAEM5:Myeloid/lymphoid neoplasm with JAK2 rearrangement
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Additional Information
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References
- ↑ Bj, Bain (2010). "Myeloid and Lymphoid Neoplasms With Eosinophilia and Abnormalities of PDGFRA, PDGFRB or FGFR1". doi:10.3324/haematol.2009.021675. PMC 2864371. PMID 20442440.CS1 maint: PMC format (link)
- ↑ Da, Arber; et al. (2016). "The 2016 Revision to the World Health Organization Classification of Myeloid Neoplasms and Acute Leukemia". PMID 27069254.
- ↑ Bain BJ, et al., (2017). Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB or FGFR1, or with PCM1-JAK2, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p72-79.
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