La tunisie Medicale - 2022 ; Vol 100 ( n°08 ) : 647-651
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Summary

Introduction : Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a proliferation of plasmacytoid cell precursors. It is a rare and difficult-to-diagnose hematological malignancy with a poor outcome.
Case presentation: We report three cases of BPDCN diagnosed in patients of different nationalities (Tunisian, Algerian and Libyan) and varying ages (eight, 65 and 74 years old). Cutaneous involvement was present in all three cases. Cytology was inconclusive in the first case, in favor of acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML) in the second and third case respectively. The diagnosis was retained by flow cytometry, highlighting the Cluster of Differentiation (CD) 4 + CD56 + phenotype of the blast population.
Conclusion: These observations illustrate diagnosis challenges, the importance of biological/clinical confrontation in order not to misdiagnose this entity. Flow cytometry is an essential diagnostic tool.

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Reference
  1. Facchetti F, Ungari M, Marocolo D, Lonardi S, Vermi W. Blastic plasmacytoid dendritic cell neoplasm. Hematol Meet Rep Former Haematol Rep [Internet]. 2009 Jun 12 [cited 2020 Jul 6];3(3). Available from: https://www.pagepress.org/journals/index.php/hmr/article/view/553
  2. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391–405.
  3. Deconinck E, Petrella T, Garnache Ottou F. Blastic Plasmacytoid Dendritic Cell Neoplasm. Hematol Oncol Clin North Am. 2020;34:491–500.
  4. Gagnier JJ, Kienle G, Altman DG, Moher D, Sox H, Riley D, et al. The CARE guidelines: consensus-based clinical case reporting guideline development. J Med Case Reports. 2013;7:223.
  5. SL-401 in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia - Full Text View - ClinicalTrials.gov [Internet]. [cited 2022 Jul 26]. Available from: https://clinicaltrials.gov/ ct2/show/NCT02113982
  6. Disseminated blastic plasmacytoid dendritic cell neoplasm. 2015 Blood;126:558.
  7. Garnache-Ottou F, Vidal C, Biichlé S, Renosi F, Poret E, Pagadoy M, et al. How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients? Blood Adv. 2019;3:4238–51.
  8. Deotare U, Yee KWL, Le LW, Porwit A, Tierens A, Musani R, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10-Color flow cytometry diagnosis and HyperCVAD therapy: BPDCN Diagnosis and Therapy. Am J Hematol. 2016;91:283–6.
  9. Julia F, Petrella T, Beylot-Barry M, Bagot M, Lipsker D, Machet L, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol. 2013;169:579–86.
  10. Momoi A, Toba K, Kawai K, Tsuchiyama J, Suzuki N, Yano T, et al. Cutaneous lymphoblastic lymphoma of putative plasmacytoid dendritic cell-precursor origin: two cases. Leuk Res. 2002;26:693–8.
  11. Bueno C, Almeida J, Lucio P, Marco J, Garcia R, de Pablos JM, et al. Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies. Haematologica. 2004;89:58–69.
  12. Tsagarakis NJ, Kentrou NA, Papadimitriou KA, Pagoni M, Kokkini G, Papadaki H, et al. Acute lymphoplasmacytoid dendritic cell (DC2) leukemia: Results from the Hellenic Dendritic Cell Leukemia Study Group. Leuk Res. 2010 ;34:438–46.
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