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
. 2023 Jan 26;17(1):39.
doi: 10.1186/s13256-022-03722-y.

Myeloid/lymphoid neoplasm with eosinophilia and BCR/FGFR1 rearrangement with transformation to cortical T-lymphoblastic lymphoma and erythroid precursors: a case report

Alejandro Pineda Isaza  1 , Santiago Castaño Quintero  2 , Lisceth Paola Quintero González  2 , Fabián Emiliano Ahumada Córdoba  3 , Andrés Felipe Arbeláez Olivar  3 , Juan Carlos Bravo Ocaña  4
Affiliations
Case Reports

Myeloid/lymphoid neoplasm with eosinophilia and BCR/FGFR1 rearrangement with transformation to cortical T-lymphoblastic lymphoma and erythroid precursors: a case report

Alejandro Pineda Isaza et al. J Med Case Rep. .

Abstract

Background: Myeloproliferative neoplasms are a group of diseases with diverse biological and clinical characteristics. As a provisional separate entity, myeloid/lymphoid neoplasms with eosinophilia and genetic rearrangement have been described, which may present an initial clinical behavior of myeloproliferation and be characterized by varied genetic rearrangements. One of these entities is associated with FGFR1 rearrangements, characterized by its low prevalence and few treatment options.

Case presentation: We present the case of a 53-year-old Mestizo male patient of Hispanic origin who initially presented weight loss and fatigue, with a complete blood count showing leukocytosis and eosinophilia, with an initial diagnosis of nonspecific myeloproliferative disorder. In a next-generation sequencing study, BCR::FGFR1 rearrangement was documented, a diagnosis of myeloid/lymphoid neoplasia with eosinophilia and BCR::FGFR1 rearrangement was made, and hydroxyurea therapy was initiated. Subsequently, transformation to cortical T-lymphoblastic leukemia/lymphoma and erythroid precursors was documented, requiring management with chemotherapy.

Conclusions: Myeloid/lymphoid neoplasms with eosinophilia and genetic rearrangements constitute a group of deeply heterogeneous diseases with variable clinical and diagnostic characteristics and whose treatment is not clearly defined.

Keywords: Eosinophilia; FGFR1 rearrangement; Myeloid/lymphoid neoplasms; Precursor T-cell lymphoblastic leukemia–lymphoma.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Splenic tissue samples demonstrating cellular proliferation with a histological pattern that has a distribution of sheets of mature eosinophils with its precursors and are accompanied with other big cells distributed in nests, with immature chromatin nuclei, nucleoles, and scarce cytoplasm (A and B). Lymphatic node samples evidencing infiltration by immature cells described previously at the spleen, which comprise immature chromatin nuclei with abundant eosinophils and mastocytes (arrowhead). Furthermore, different lymph node zones are infiltrated by lymphoblastic cells with diffuse growth pattern (arrow) (C)
Fig. 2
Fig. 2
Population infiltrating the node corresponds to cortical T lymphoblasts, which are positive for TdT (A), CD1a (B), CD3 (C), CD4, CD8, CD99, CD5, CD7, and BCL2. Neoplastic population of positive erythroid precursors is found positive for E-cadherin (D), glycophorin (E), and heterogeneous weak expression of CD117. The cellular proliferation index calculated with ki67 is 95%
See this image and copyright information in PMC

References

    1. Spivak J. Myeloproliferative neoplasms. N Engl J Med. 2017;376:2168–2181. doi: 10.1056/NEJMra1406186. - DOI - PubMed
    1. Vardiman J. Myeloproliferative Neoplasm. En: Jaffe E et al. Hematopathology 2d Ed. Elsevier, 2017.
    1. Shallis R, et al. Epidemiology of the classical myeloproliferative neoplasms: the four corners of an expansive and complex map. Blood Rev. 2020;42:100706. doi: 10.1016/j.blre.2020.100706. - DOI - PubMed
    1. Strati P, et al. Myeloid/lymphoid neoplasms with FGFR1 rearrangement. Leuk Lymphoma. 2018;59(7):1672–1676. doi: 10.1080/10428194.2017.1397663. - DOI - PubMed
    1. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Revised 4th ed. 2016; Ch 11.

Publication types

MeSH terms

Substances