Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy. It was previously termed as blastic natural-killer lymphoma or agranular CD4+ natural killer cell leukemia.
It only represent a very small proportion (~0.44%) of all hematological malignancies 4. There may be a slight male predilection.
It often presents in the skin. Clinical course can be aggressive in adults and apparently milder in children. Some patient may have a leukemic presentation 5.
Histologically, the tumor cells may be either blastoid or pleomorphic. They express several markers of plasmacytoid dendritic cells which include:
Lymphadenopathy and hepatosplenomegaly may be present.
Skin lesions and lymph node metastases may appear hypermetabolic on 18F-FDG PET imaging. Positive bone marrow involvement, however, may not be always evident on 18F-FDG PET 4.
Treatment and prognosis
Chemotherapy is the preferred treatment, along with allogeneic stem cell transplantation. Patients usually have a poor outcome with a median survival reported around 12-14 months. Advanced stage and older age may imply poorer prognosis 4.
- 1. Lucioni M, Novara F, Fiandrino G, Riboni R, Fanoni D, Arra M, Venegoni L, Nicola M, Dallera E, Arcaini L, Onida F, Vezzoli P, Travaglino E, Boveri E, Zuffardi O, Paulli M, Berti E. Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion. Blood. 118 (17): 4591-4. doi:10.1182/blood-2011-03-337501 - Pubmed
- 2. Pagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F, Lunghi M, Pica G, Onida F, Cattaneo C, Piccaluga PP, Di Bona E, Todisco E, Musto P, Spadea A, D'Arco A, Pileri S, Leone G, Amadori S, Facchetti F. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica. 98 (2): 239-46. doi:10.3324/haematol.2012.072645 - Pubmed
- 3. Riaz W, Zhang L, Horna P, Sokol L. Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy. Cancer control : journal of the Moffitt Cancer Center. 21 (4): 279-89. Pubmed
- 4. Jeong D, Choi JW, Jeong K, Sokol L. CT findings associated with blastic plasmacytoid dendritic cell neoplasm: a case report. Acta radiologica open. 5 (7): 2058460116657688. doi:10.1177/2058460116657688 - Pubmed
- 5. Rauh MJ, Rahman F, Good D, Silverman J, Brennan MK, Dimov N, Liesveld J, Ryan DH, Burack WR, Bennett JM. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review. Leukemia research. 36 (1): 81-6. doi:10.1016/j.leukres.2011.07.033 - Pubmed