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Clinical Indications for PDGFRA, PDGFRB, or FGFR1 Testing in Hematologic Neoplasms
EP25387
Poster Title: Clinical Indications for PDGFRA, PDGFRB, or FGFR1 Testing in Hematologic Neoplasms
Submitted on 08 Feb 2017
Author(s): Lynh Nguyen MD, Jinming Song MD PhD, and Ling Zhang MD
Affiliations: H. Lee Moffitt Cancer Center
This poster was presented at USF Health Research Day 2017
Poster Views: 1,537
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Poster Information
Abstract: Eosinophilia encompasses a broad range of disorders, both reactive and neoplastic. Neoplastic proliferation of eosinophils can be associated with myeloid or lymphoid disorders or both. Disease prognosis relies on accurately classifying the eosinophilia.

The World Health Organization (WHO) established a molecular classification scheme of disease subtypes including myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1, chronic eosinophilic leukemia, not otherwise specified (CEL, NOS), acute myeloid leukemia with increased eosinophils (AML eos), and idiopathic hypereosinophilic syndrome (HES).

Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1 are extremely rare and clinically and histologically heterogeneous. They can manifest as a myeloproliferative neoplasm (MPN), acute myeloid or lymphoblastic leukemia (AML or ALL), or chronic myelomonocytic leukemia (CMML). Clinically the neoplasm can resemble chronic eosinophilic leukemia (CEL, NOS) or idiopathic hypereosinophilic syndrome (HES).

In contrast to FGFR1 related neoplasms, those with PDGFRA or PDGFRB rearrangements often respond well to tyrosine kinase inhibitor therapy (TKI), emphasizing the importance of cytogenetic and molecular genetic analysis in identifying these patients; whereas patients with neoplasms associated with FGFR1 rearrangements usually do not respond.

Specific indications to perform PDGFRA, PDGFRB or FGFR1 analysis by fluorescent in situ hybridization (FISH) or by karyotyping have not been established as eosinophilia may be absent in a subset of cases and is therefore not a reliable indicator for laboratory genetic testing.
Summary: Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1 are rare and clinically heterogeneous. In contrast to FGFR1 related neoplasms, those with PDGFRA or PDGFRB rearrangements often respond well to treatment, emphasizing the importance of cytogenetic and molecular genetic analysis in identifying these patients. However, specific indications for such testing have not been established and eosinophilia is not a reliable indicator.References: 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.

Swerdlow SH, Campo E, Harris NL, et al. (Eds). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon 2008.
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