Blastic Plasmacytoid Dendritic Cell Neoplasm in a Young Female: Rare and Challenging

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic malignancy with an aggressive behavior, seen commonly in the elderly age group and usually involves the skin. Evaluation, diagnosis, and treatment pose unique challenges with poor outcomes. We report a case of an 18-year-old female who presented with lymphadenopathy and bicytopenia. The diagnosis of BPDCN was an initial challenge. She thereafter received intensive chemotherapy followed by an allogenic partially mismatched sibling donor hematopoietic cell transplantation, only to relapse in a few months. Beginning from the diagnosis, the management of this rare disorder and its relapse until her last followup provided several learning opportunities.

Keywords

Blastic plasmacytoid dendritic cell neoplasm - CD123 - hematopoietic cell transplant

• References

• 1 Kerr 2nd D, Zhang L, Sokol L. Blastic plasmacytoid dendritic cell neoplasm. Curr Treat Options Oncol 2019; 20: 9
• 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 Reimer P, Rüdiger T, Kraemer D, Kunzmann V, Weissinger F, Zettl A. et al. What is CD4+CD56+malignancy and how should it be treated?. Bone Marrow Transplant 2003; 32: 637-46
• 4 Żychowska M, Batycka-Baran A, Woźniak Z, Maj J. Blastic plasmacytoid dendritic cell neoplasm: A rare lymphoma of extremely aggressive course. Postepy Dermatol Alergol 2017; 34: 504-6
• 5 Martín-Martín L, López A, Vidriales B, Caballero MD, Rodrigues AS, Ferreira SI. et al. Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile. Oncotarget 2015; 6: 19204-16
• 6 Sullivan JM, Rizzieri DA. Treatment of blastic plasmacytoid dendritic cell neoplasm. Hematology Am Soc Hematol Educ Program 2016; 2016: 16-23
• 7 Feuillard J, Jacob MC, Valensi F, Maynadié M, Gressin R, Chaperot L. et al. Clinical and biologic features of CD4(+) CD56(+) malignancies. Blood 2002; 99: 1556-63
• 8 Pagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F. et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: An Italian multicenter study. Haematologica 2013; 98: 239-46
• 9 Pemmaraju N, Thomas DA, Kantarjian H, Duvic M, Khoury JD, Patel K. et al. Blastic plasmacytoid dendritic cell neoplasm (BPDCN): A large single-center experience: Analysis of clinical and molecular characteristics and patient outcomes. Blood 2015; 126: 3746
• 10 Male HJ, Davis MB, McGuirk JP, Abhyankar S, Aljitawi OS, Zhang D. et al. Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission. Int J Hematol 2010; 92: 398-400