Blastic plasmacytoid dendritic cell neoplasm: case report and literature review
Authors:
M. Čerňan 1; T. Szotkowski 1; Z. Rusiňáková 1; L. Raida 1; M. Tichý 2; M. Dušková 2
Authors‘ workplace:
Hemato-onkologická klinika LF UP a FN Olomouc
1; Ústav klinické a molekulární patologie LF UP a FN Olomouc
2
Published in:
Transfuze Hematol. dnes,23, 2017, No. 3, p. 134-144.
Category:
Comprehensive Reports, Original Papers, Case Reports
Overview
Blastic plasmacytoid dendritic cell neoplasm represents a rare hematologic malignancy of aggressive biology and unfavourable prognosis. This disease is characterised by primary skin lesions often followed by bone marrow and extramedullary involvement. Diagnosis is based on immunohistochemistry and flow cytometric demonstration of CD4 and CD56 co-expression with variable positivity of other markers specific to dendritic cells. The median age at diagnosis ranges from 60 to 70 years, with males affected 3 times more frequently. Knowledge of its treatment is limited to retrospective analyses of small patient cohorts. Despite generally favourable treatment response to chemotherapy, the disease tends to relapse in a few months with rapid systemic dissemination. The reported data suggest that induction therapy for acute lymphoblastic leukaemia followed by allogeneic hematopoietic cell transplantation is more effective than other induction or consolidation chemotherapy strategies.
The article reports the case of a 22-year old male with newly diagnosed blastic plasmacytoid dendritic cell neoplasm with skin-limited symptoms. The patient underwent induction and one cycle of consolidation chemotherapy accordingto the GMALL protocol for acute lymphoblastic leukaemia followed by allogeneic hematopoietic cell transplantation in the first complete remission. Chemotherapy and transplantation were not associated with serious complications and the patient remains in remission with a good quality of life, 22 months after allogeneic transplantation. This case details the successful treatment strategy in a disease with unfavourable prognosis. The article also presents a review of the literature regarding diagnosis, clinical presentation and treatment strategies in patients with blastic plasmacytoid dendritic cell neoplasm. The aim of the paper is also to provide information about this rare nosological unit and promote prompt diagnosis and treatment initiation necessary for disease control.
KEY WORDS:
blastic plasmacytoid dendritic cell neoplasm – BPDCN – induction chemotherapy – GMALL – bone marrow transplantation – immunophenotyping
Sources
1. Pagano L, Valentini CG, Grammatico S, Pulsoni A. Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches. Br J Haematol 2016;174(2):188–202.
2. Adachi M, Maeda K, Takekawa M, et al. High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol 1994;47(4):278–282.
3. Brody JP, Allen S, Schulman P, et al. Acute agranular CD4-positive natural killer cell leukemia. Comprehensive clinicopathologic studies including virologic and in vitro culture with inducing agents. Cancer 1995;75(10):2474–2483.
4. Petrella T, Comeau MR, Maynadié M, et al. ‚Agranular CD4+ CD56+ hematodermic neoplasm‘ (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes. Am J Surg Pathol 2002;26(7):852–862.
5. Vardiman JW, Thiele J, Arber DA, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 2009;114:937–951.
6. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127:2391–2405.
7. Jacob MC, Chaperot L, Mossuz P, et al. CD4+ CD56+ lineage negative malignancies: a new entity developed from malignant early plasmacytoid dendritic cells. Haematologica 2003;88(8):941–955.
8. Bueno C, Almeida J, Lucio P, et al. Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies. Haematologica 2004;89(1):58–69.
9. Petrella T, Bagot M, Willemze R, et al. Blastic NK-cell lymphomas (agranular CD4+CD56+ hematodermic neoplasms): a review. Am J Clin Pathol 2005;123(5):662–675.
10. Facchetti F, Cigognetti M, Fisogni S, et al. Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol 2016;29(2):98–111.
11. Jegalian AG, Buxbaum NP, Facchetti F, et al. Blastic plasmacytoid dendritic cell neoplasm in children: diagnostic features and clinical implications. Haematologica 2010;95(11):1873–1879.
12. Pagano L, Valentini CG, Pulsoni A, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica 2013;98(2):239–246.
13. Tsagarakis NJ, Kentrou NA, Papadimitriou KA, et al. Acute lymphoplasmacytoid dendritic cell (DC2) leukemia: results from the Hellenic Dendritic Cell Leukemia Study Group. Leuk Res 2010;34(4):438–446.
14. Julia F, Petrella T, Beylot-Barry M, et al. Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol 2013;169(3):579–586.
15. Leroux D, Mugneret F, Callanan M, et al. CD4(+), CD56(+) DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Français de Cytogénétique Hématologique. Blood 2002;99(11):4154–4159.
16. Lucioni M, Novara F, Fiadrino G, et al. Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion. Blood 2011;118:4591–4594.
17. Taylor J, Kim SS, Stevenson KE, et al. Loss-of-function mutations in the splicing factor ZRSR2 are common in blastic plasmacytoid dendritic cell neoplasm and have male predominance. Blood 2013;122:741.
18. Reimer P, Rüdiger T, Kraemer D, et al. sWhat is CD4+CD56+ malignancy and how should it be treated? Bone Marrow Transplant 2003;32(7):637– 646.
19. Aoki T, Suzuki R, Kuwatsuka Y, et al. Long-term survival following autologous and allogeneic stem cell transplantation for blastic plasmacytoid dendritic cell neoplasm. Blood 2015;125(23):3559–3562.
20. Dalle S, Beylot-Barry M, Bagot M, et al. Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? Br J Dermatol 2010;162(1):74–79.
21. Poret E, Vidal Ch, Desbrosses Y, et al. How to treat blastic plasmacytoid dendritic cell neoplasm (BPDCN) patients: results on 86 patients of the French BPDCN Network. Blood 2015;126:456.
22. Pemmaraju N, Thomas D, Kantarjian HM, et al. Analysis of Outcomes of Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm. Blood 2012;120:3554.
23. Kharfan-Dabaja MA, Lazarus HM, Nishihori T, Mahfouz RA, Hamadani M. Diagnostic and therapeutic advances in blastic plasmacytoid dendritic cell neoplasm: a focus on hematopoietic cell transplantation. Biol Blood Marrow Transplant 2013;19(7):1006–1012.
24. Frankel AE, Woo JH, Ahn C, et al. Activity of SL-401, a targeted therapy directed to interleukin-3 receptor, in blastic plasmacytoid dendritic cell neoplasm patients. Blood 2014;124:385–392.
25. Stein EM, Tallman MS. Emerging therapeutic drugs for AML. Blood 2016;127:71–78.
26. Pevná M, Kissová J, Doubek M, Adam Z, Klabusay M, et al. Leukemie z dendritických buněk CD4+56+, typ DC2. Vnitř Lék 2010;56(Suppl 2):183–187.
27. Klabusay M, Pevná M, Kissová J, et al. Vzácná diagnóza: CD4+56+ leukémie z dendritických buněk typu DC2. Čas Lék Čes 2008;147:511–515.
Labels
Haematology Internal medicine Clinical oncologyArticle was published in
Transfusion and Haematology Today
2017 Issue 3
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