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Case Reports
. 2021 Oct 27;7(4):57-62.
doi: 10.22551/2020.29.0704.10174. eCollection 2020.

A very rare case of FLT3-D835 positive blastic plasmacytoid dendritic cell neoplasm

Vlad Andrei Cianga  1   2 , Cătălin Doru Dănăilă  1   2 , Ion Antohe  2 , Raluca Oană  3 , Mihaela Mențel  4 , Iuliu Ivanov  5 , Loredana Dragoș  5 , Angela Smaranda Dăscălescu  1   2
Affiliations
Case Reports

A very rare case of FLT3-D835 positive blastic plasmacytoid dendritic cell neoplasm

Vlad Andrei Cianga et al. Arch Clin Cases. .

Abstract

Blastic plasmacytoid dendritic cell neoplasms (BPDCNs) are extremely rare and aggressive hematological malignancies that derive from precursors of plasmacytoid dendritic cells (pDC) and frequently involve skin lesions and bone marrow infiltration. They mostly affect the elderly population and the prognosis is poor with the therapeutic choices currently available. Diagnosis is made with the help of tools such as immunohistochemistry and flow cytometry. Here, we present a particular case of BPDCN with a positive FLT3-D835 mutation and we discuss the possible impact this may have on the evolution of the disease and response to treatment.

Keywords: FLT3-D835; acute leukemia; aggressive hematopoietic neoplasm; blastic plasmacytoid dendritic cell neoplasm; flow cytometry.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
A, B. Morphology analysis of the BM cells reveals a population of 27% medium sized blast cells that present irregular nuclear outlines, fine chromatin and reduced agranular basophilic cytoplasm.
Fig. 2
Fig. 2
Flow cytometry was performed on the BM cells. The blastic plasmacytoid dendritic cell population is highlighted in orange. The blasts were intermediate positive for CD45 (a); partially positive for CD117 and CD7 (b, c); positive for CD4, CD36, CD56, CD123, HLA-DR and NG2 (d, e, f, g, h, i) and negative for CD94 (f), CD34 (j), cyCD3 and cyCD79a (k) and cyMPO (l).
See this image and copyright information in PMC

References

    1. Faccheti F, Petrella T, Pileri SA. Blastic Plasmacytoid dendritic cell neoplasm. In: Swerdlow SH, editor. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition) Vol. 9. IARC: Lyon; 2017. pp. 174–177.
    1. O’Keeffe M, Mok WH, Radford KJ. Human dendritic cell subsets and function in health and disease. Cell Mol Life Sci. 2015;72(22):1–17. doi: 10.1007/s00018-015-2005-0. - DOI - PMC - PubMed
    1. Hermans IF, Silk JD, Gileadi U, et al. NKT cells enhance CD4+ and CD8+ T cell responses to soluble antigen in vivo through direct interaction with dendritic cells. J Immunol. 2003;171(10):5140–5147. doi: 10.4049/jimmunol.171.10.5140. - DOI - PubMed
    1. Reizis B, Bunin A, Ghosh HS, Lewis KL, Sisirak V. Plasmacytoid dendritic cells: recent progress and open questions. Annu Rev Immunol. 2011;29:163–183. doi: 10.1146/annurev-immunol-031210-101345. - DOI - PMC - PubMed
    1. Venugopal S, Zhou S, El Jamal SM, Lane AA, Mascarenhas J. Blastic Plasmacytoid Dendritic Cell Neoplasm-Current Insights. Clin Lymphoma Myeloma Leuk. 2019;19(9):545–554. doi: 10.1016/j.clml.2019.06.002. - DOI - PubMed

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