Skip to main content

Advertisement

SpringerLink
Log in

Low-dose venetoclax combined with azacitidine for blastic plasmacytoid dendritic cell neoplasm: a case report and literature review

  • Case Report
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that is highly aggressive with a poor prognosis. There is no standard treatment for BPDCN. Although conventional chemotherapies are usually sensitive in the initial therapy, relapse and drug resistance are inevitable within a short duration. Targeted therapies have enlightened new prospects for the treatment of BPDCN, especially for those in a frail state and intolerable to standard chemotherapies or hematopoietic stem cell transplantation. Here, we report an 82-year-old man diagnosed with cutaneous-limited BPDCN. Considering the old age and limited involvement of the tumor, we reduced the dosage of venetoclax. His skin lesions subsided significantly after 1 cycle of azacytidine (100 mg d1-7) combined with reduced doses of venetoclax (200 mg d1-14). The reduction in the dose of venetoclax avoided severe myelosuppression while achieving satisfactory outcomes. The patient received 2 cycles of therapy with no skin lesions re-occurred for 7 months before relapsing.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Data availability

No datasets were generated or analysed during the current study.

References

  1. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW (2016) The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 127(20):2391–2405. https://doi.org/10.1182/blood-2016-03-643544

    Article  CAS  PubMed  Google Scholar 

  2. Molina Castro D, Perilla Suarez O, Cuervo-Sierra J, Moreno A (2022) Blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement: a case report. Cureus 14(4):e23888. https://doi.org/10.7759/cureus.23888

    Article  PubMed  PubMed Central  Google Scholar 

  3. 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, Gimema A (2013) Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. Haematologica 98(2):239–246. https://doi.org/10.3324/haematol.2012.072645

    Article  PubMed  PubMed Central  Google Scholar 

  4. Pemmaraju N, Wilson NR, Khoury JD, Jain N, Daver N, Pierce S, Jabbour E, Kadia T, DiNardo C, Garcia-Manero G, Qazilbash M, Konopleva M, Kantarjian H (2021) Central nervous system involvement in blastic plasmacytoid dendritic cell neoplasm. Blood 138(15):1373–1377. https://doi.org/10.1182/blood.2021011817

    Article  CAS  PubMed  Google Scholar 

  5. Li Y, Li Z, Lin HL, Chen XH, Li B (2011) Primary cutaneous blastic plasmacytoid dendritic cell neoplasm without extracutaneous manifestation: case report and review of the literature. Pathol Res Pract 207(1):55–59. https://doi.org/10.1016/j.prp.2010.05.008

    Article  PubMed  Google Scholar 

  6. Pileri A, Delfino C, Grandi V, Agostinelli C, Pileri SA, Pimpinelli N (2012) Blastic plasmacytoid dendritic cell neoplasm (BPDCN): the cutaneous sanctuary. G Ital Dermatol Venereol 147(6):603–608

    CAS  PubMed  Google Scholar 

  7. Garnache-Ottou F, Vidal C, Biichle S, Renosi F, Poret E, Pagadoy M, Desmarets M, Roggy A, Seilles E, Soret L, Schillinger F, Puyraimond S, Petrella T, Preudhomme C, Roumier C, MacIntyre EA, Harrivel V, Desbrosses Y, Gruson B, Genevieve F, Thepot S, Drebit Y, Leguay T, Gros FX, Lechevalier N, Saussoy P, Salaun V, Cornet E, Benseddik Z, Veyrat-Masson R, Wagner-Ballon O, Salanoubat C, Maynadie M, Guy J, Caillot D, Jacob MC, Cahn JY, Gressin R, Rose J, Quesnel B, Guerin E, Trimoreau F, Feuillard J, Gourin MP, Plesa A, Baseggio L, Arnoux I, Vey N, Blaise D, Lacroix R, Arnoulet C, Benet B, Dorvaux V, Bret C, Drenou B, Debliquis A, Latger-Cannard V, Bonmati C, Bene MC, Peterlin P, Ticchioni M, Rohrlich PS, Arnaud A, Wickenhauser S, Bardet V, Brechignac S, Papoular B, Raggueneau V, Vargaftig J, Letestu R, Lusina D, Braun T, Foissaud V, Tamburini J, Bennani H, Freynet N, Cordonnier C, Le Garff-Tavernier M, Jacques N, Maloum K, Roos-Weil D, Bouscary D, Asnafi V, Lhermitte L, Suarez F, Lengline E, Feger F, Battipaglia G, Mohty M, Bouyer S, Ghoual O, Dindinaud E, Basle C, Puyade M, Lafon C, Fest T, Roussel M, Cahu X, Bera E, Daliphard S, Jardin F, Campos L, Solly F, Guyotat D, Galoisy AC, Eischen A, Mayeur-Rousse C, Guffroy B, Recher C, Loosveld M, Garnier A, Barlogis V, Rosenthal MA, Brun S, Contentin N, Maury S, Callanan M, Lefebvre C, Maillard N, Okamba P, Ferrand C, Adotevi O, Saas P, Angelot-Delettre F, Binda D, Deconinck E (2019) How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients? Blood Adv 3(24):4238–4251. https://doi.org/10.1182/bloodadvances.2019000647

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Poussard M, Angelot-Delettre F, Deconinck E (2022) Conventional therapeutics in BPDCN patients-do they still have a place in the era of targeted therapies? Cancers (Basel) 14(15). https://doi.org/10.3390/cancers14153767

  9. Kerr D 2nd, Zhang L, Sokol L (2019) Blastic plasmacytoid dendritic cell neoplasm. Curr Treat Options Oncol 20(1):9. https://doi.org/10.1007/s11864-019-0605-x

    Article  PubMed  Google Scholar 

  10. DiNardo CD, Rausch CR, Benton C, Kadia T, Jain N, Pemmaraju N, Daver N, Covert W, Marx KR, Mace M, Jabbour E, Cortes J, Garcia-Manero G, Ravandi F, Bhalla KN, Kantarjian H, Konopleva M (2018) Clinical experience with the BCL2-inhibitor venetoclax in combination therapy for relapsed and refractory acute myeloid leukemia and related myeloid malignancies. Am J Hematol 93(3):401–407. https://doi.org/10.1002/ajh.25000

    Article  CAS  PubMed  Google Scholar 

  11. Montero J, Stephansky J, Cai T, Griffin GK, Cabal-Hierro L, Togami K, Hogdal LJ, Galinsky I, Morgan EA, Aster JC, Davids MS, LeBoeuf NR, Stone RM, Konopleva M, Pemmaraju N, Letai A, Lane AA (2017) Blastic plasmacytoid dendritic cell neoplasm is dependent on BCL2 and sensitive to venetoclax. Cancer Discov 7(2):156–164. https://doi.org/10.1158/2159-8290.CD-16-0999

    Article  CAS  PubMed  Google Scholar 

  12. Pollyea DA, Altman JK, Assi R, Bixby D, Fathi AT, Foran JM, Gojo I, Hall AC, Jonas BA, Kishtagari A, Lancet J, Maness L, Mangan J, Mannis G, Marcucci G, Mims A, Moriarty K, Mustafa Ali M, Neff J, Nejati R, Olin R, Percival ME, Perl A, Przespolewski A, Rao D, Ravandi F, Shallis R, Shami PJ, Stein E, Stone RM, Sweet K, Thota S, Uy G, Vachhani P, Cassara CJ, Freedman-Cass DA, Stehman K (2023) Acute myeloid leukemia, version 3.2023, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw 21(5):503–513. https://doi.org/10.6004/jnccn.2023.0025

    Article  CAS  PubMed  Google Scholar 

  13. Khoury JD, Solary E, Abla O, Akkari Y, Alaggio R, Apperley JF, Bejar R, Berti E, Busque L, Chan JKC, Chen W, Chen X, Chng WJ, Choi JK, Colmenero I, Coupland SE, Cross NCP, De Jong D, Elghetany MT, Takahashi E, Emile JF, Ferry J, Fogelstrand L, Fontenay M, Germing U, Gujral S, Haferlach T, Harrison C, Hodge JC, Hu S, Jansen JH, Kanagal-Shamanna R, Kantarjian HM, Kratz CP, Li XQ, Lim MS, Loeb K, Loghavi S, Marcogliese A, Meshinchi S, Michaels P, Naresh KN, Natkunam Y, Nejati R, Ott G, Padron E, Patel KP, Patkar N, Picarsic J, Platzbecker U, Roberts I, Schuh A, Sewell W, Siebert R, Tembhare P, Tyner J, Verstovsek S, Wang W, Wood B, Xiao W, Yeung C, Hochhaus A (2022) The 5th edition of the World Health Organization classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms. Leukemia 36(7):1703–1719. https://doi.org/10.1038/s41375-022-01613-1

    Article  PubMed  PubMed Central  Google Scholar 

  14. Albiol N, Novelli S, Mozos A, Pratcorona M, Martino R, Sierra J (2021) Venetoclax in relapsed/refractory blastic plasmacytoid dendritic cell neoplasm with central nervous system involvement: a case report and review of the literature. J Med Case Rep 15(1):326. https://doi.org/10.1186/s13256-021-02939-7

    Article  PubMed  PubMed Central  Google Scholar 

  15. Deotare U, Yee KW, Le LW, Porwit A, Tierens A, Musani R, Barth D, Torlakovic E, Schimmer A, Schuh AC, Seftel M, Minden MD, Gupta V, Hyjek E (2016) Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10-color flow cytometry diagnosis and HyperCVAD therapy. Am J Hematol 91(3):283–286. https://doi.org/10.1002/ajh.24258

    Article  CAS  PubMed  Google Scholar 

  16. Wilson NR, Konopleva M, Khoury JD, Pemmaraju N (2021) Novel Therapeutic approaches in blastic plasmacytoid dendritic cell neoplasm (BPDCN): era of targeted therapy. Clin Lymphoma Myeloma Leuk 21(11):734–740. https://doi.org/10.1016/j.clml.2021.05.018

    Article  CAS  PubMed  Google Scholar 

  17. Economides MP, McCue D, Lane AA, Pemmaraju N (2019) Tagraxofusp, the first CD123-targeted therapy and first targeted treatment for blastic plasmacytoid dendritic cell neoplasm. Expert Rev Clin Pharmacol 12(10):941–946. https://doi.org/10.1080/17512433.2019.1662297

    Article  CAS  PubMed  Google Scholar 

  18. Lane AA (2020) Novel therapies for blastic plasmacytoid dendritic cell neoplasm. Hematol Oncol Clin North Am 34(3):589–600. https://doi.org/10.1016/j.hoc.2020.01.007

    Article  PubMed  Google Scholar 

  19. Wierda WG, Tambaro FP (2020) How I manage CLL with venetoclax-based treatments. Blood 135(17):1421–1427. https://doi.org/10.1182/blood.2019002841

    Article  PubMed  Google Scholar 

  20. Pollyea DA, Amaya M, Strati P, Konopleva MY (2019) Venetoclax for AML: changing the treatment paradigm. Blood Adv 3(24):4326–4335. https://doi.org/10.1182/bloodadvances.2019000937

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Pemmaraju N, Konopleva M, Lane AA (2019) More on blastic plasmacytoid dendritic-cell neoplasms. N Engl J Med 380(7):695–696. https://doi.org/10.1056/NEJMc1814963

    Article  PubMed  Google Scholar 

  22. Julia F, Petrella T, Beylot-Barry M, Bagot M, Lipsker D, Machet L, Joly P, Dereure O, Wetterwald M, d’Incan M, Grange F, Cornillon J, Tertian G, Maubec E, Saiag P, Barete S, Templier I, Aubin F, Dalle S (2013) Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients. Br J Dermatol 169(3):579–586. https://doi.org/10.1111/bjd.12412

    Article  CAS  PubMed  Google Scholar 

  23. Deconinck E, Petrella T, GarnacheOttou F (2020) Blastic plasmacytoid dendritic cell neoplasm: clinical presentation and diagnosis. Hematol Oncol Clin North Am 34(3):491–500. https://doi.org/10.1016/j.hoc.2020.01.010

    Article  PubMed  Google Scholar 

Download references

Funding

This study was funded by grants from the National Natural Science Foundation of China (No. 82170123).

Author information

Author notes

  1. Qiuyan Wang and Yajing Zhao contributed equally to this work.

Authors and Affiliations

  1. Department of Hematology, Qilu Hospital of Shandong University Dezhou Hospital, Dezhou, Shandong, China

    Qiuyan Wang

  2. Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, 107 West Wenhua Road, Jinan, 250012, Shandong, China

    Yajing Zhao, Qi Feng, Xin Li, Wen Wang, Xiaoyuan Dong, Xinguang Liu, Jun Peng & Chuanfang Liu

  3. Shandong Provincial Key Laboratory of Immunohematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, 107 West Wenhua Road, Jinan, 250012, Shandong, China

    Yajing Zhao, Qi Feng, Xin Li, Wen Wang, Xiaoyuan Dong, Xinguang Liu, Jun Peng & Chuanfang Liu

  4. Shandong Provincial Hospital for Skin Diseases, Shandong First Medical University, Jinan, Shandong, China

    Xiao Zang, Guizhi Zhou & Yongxia Liu

  5. Shandong Provincial Institute of Dermatology and Venereology, Shandong Academy of Medical Sciences, Jinan, Shandong, China

    Xiao Zang, Guizhi Zhou & Yongxia Liu

  6. Advanced Medical Research Institute, Shandong University, Jinan, 250012, Shandong, China

    Jun Peng

Authors
  1. Qiuyan Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yajing Zhao
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Xiao Zang
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Guizhi Zhou
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Yongxia Liu
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Qi Feng
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Xin Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Wen Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Xiaoyuan Dong
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Xinguang Liu
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Jun Peng
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Chuanfang Liu
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Qiuyan Wang, Yajing Zhao, and Xiao Zang were involved in Conceptualization, Methodology, Investigation, Formal analysis, and Writing - original draft; Guizhi Zhou and Yongxia Liu made the Histopathologic diagnosis, and Conceptualization. Qi Feng, Xin Li, Wen Wang, and Xiaoyuan Dong were involved in Visualization and investigation; Chuan-fang Liu and Peng Jun were involved in Resources and Supervision; Xin-guang Liu was involved in Conceptualization, Resources, Supervision and Writing - Review & Editing.

Corresponding authors

Correspondence to Xinguang Liu or Chuanfang Liu.

Ethics declarations

Ethics statement

Our study was approved by the ethics committees of Qilu Hospital of Shandong University according to the Helsinki Principles. Written informed consent was obtained from the patient for the publication of the clinical information.

Competing interests

The authors declare no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wang, Q., Zhao, Y., Zang, X. et al. Low-dose venetoclax combined with azacitidine for blastic plasmacytoid dendritic cell neoplasm: a case report and literature review. Ann Hematol 103, 999–1005 (2024). https://doi.org/10.1007/s00277-024-05633-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-024-05633-y

Keywords

Search

Navigation