Blood Advances (Dec 2019)

How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients?

  • Francine Garnache-Ottou,
  • Chrystelle Vidal,
  • Sabeha Biichlé,
  • Florian Renosi,
  • Eve Poret,
  • Maïder Pagadoy,
  • Maxime Desmarets,
  • Anne Roggy,
  • Estelle Seilles,
  • Lou Soret,
  • Françoise Schillinger,
  • Sandrine Puyraimond,
  • Tony Petrella,
  • Claude Preudhomme,
  • Christophe Roumier,
  • Elisabeth A. MacIntyre,
  • Véronique Harrivel,
  • Yohan Desbrosses,
  • Bérengère Gruson,
  • Franck Geneviève,
  • Sylvain Thepot,
  • Yuriy Drebit,
  • Thibaut Leguay,
  • François-Xavier Gros,
  • Nicolas Lechevalier,
  • Pascale Saussoy,
  • Véronique Salaun,
  • Edouard Cornet,
  • Zehaira Benseddik,
  • Richard Veyrat-Masson,
  • Orianne Wagner-Ballon,
  • Célia Salanoubat,
  • Marc Maynadié,
  • Julien Guy,
  • Denis Caillot,
  • Marie-Christine Jacob,
  • Jean-Yves Cahn,
  • Rémy Gressin,
  • Johann Rose,
  • Bruno Quesnel,
  • Estelle Guerin,
  • Franck Trimoreau,
  • Jean Feuillard,
  • Marie-Pierre Gourin,
  • Adriana Plesa,
  • Lucile Baseggio,
  • Isabelle Arnoux,
  • Norbert Vey,
  • Didier Blaise,
  • Romaric Lacroix,
  • Christine Arnoulet,
  • Blandine Benet,
  • Véronique Dorvaux,
  • Caroline Bret,
  • Bernard Drenou,
  • Agathe Debliquis,
  • Véronique Latger-Cannard,
  • Caroline Bonmati,
  • Marie-Christine Bene,
  • Pierre Peterlin,
  • Michel Ticchioni,
  • Pierre-Simon Rohrlich,
  • Anne Arnaud,
  • Stefan Wickenhauser,
  • Valérie Bardet,
  • Sabine Brechignac,
  • Benjamin Papoular,
  • Victoria Raggueneau,
  • Jacques Vargaftig,
  • Rémi Letestu,
  • Daniel Lusina,
  • Thorsten Braun,
  • Vincent Foissaud,
  • Jérôme Tamburini,
  • Hind Bennani,
  • Nicolas Freynet,
  • Catherine Cordonnier,
  • Magali Le Garff-Tavernier,
  • Nathalie Jacques,
  • Karim Maloum,
  • Damien Roos-Weil,
  • Didier Bouscary,
  • Vahid Asnafi,
  • Ludovic Lhermitte,
  • Felipe Suarez,
  • Etienne Lengline,
  • Frédéric Féger,
  • Giorgia Battipaglia,
  • Mohamad Mohty,
  • Sabrina Bouyer,
  • Ouda Ghoual,
  • Elodie Dindinaud,
  • Caroline Basle,
  • Mathieu Puyade,
  • Carinne Lafon,
  • Thierry Fest,
  • Mikael Roussel,
  • Xavier Cahu,
  • Elsa Bera,
  • Sylvie Daliphard,
  • Fabrice Jardin,
  • Lydia Campos,
  • Françoise Solly,
  • Denis Guyotat,
  • Anne-Cécile Galoisy,
  • Alice Eischen,
  • Caroline Mayeur-Rousse,
  • Blandine Guffroy,
  • Christian Recher,
  • Marie Loosveld,
  • Alice Garnier,
  • Vincent Barlogis,
  • Maria Alessandra Rosenthal,
  • Sophie Brun,
  • Nathalie Contentin,
  • Sébastien Maury,
  • Mary Callanan,
  • Christine Lefebvre,
  • Natacha Maillard,
  • Patricia Okamba,
  • Christophe Ferrand,
  • Olivier Adotevi,
  • Philippe Saas,
  • Fanny Angelot-Delettre,
  • Delphine Binda,
  • Eric Deconinck

Journal volume & issue
Vol. 3, no. 24
pp. 4238 – 4251

Abstract

Read online

Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive leukemia for which we developed a nationwide network to collect data from new cases diagnosed in France. In a retrospective, observational study of 86 patients (2000-2013), we described clinical and biological data focusing on morphologies and immunophenotype. We found expression of markers associated with plasmacytoid dendritic cell origin (HLA-DRhigh, CD303+, CD304+, and cTCL1+) plus CD4 and CD56 and frequent expression of isolated markers from the myeloid, B-, and T-lymphoid lineages, whereas specific markers (myeloperoxidase, CD14, cCD3, CD19, and cCD22) were not expressed. Fifty-one percent of cytogenetic abnormalities impact chromosomes 13, 12, 9, and 15. Myelemia was associated with an adverse prognosis. We categorized chemotherapeutic regimens into 5 groups: acute myeloid leukemia (AML)–like, acute lymphoid leukemia (ALL)–like, lymphoma (cyclophosphamide, doxorubicin, vincristine, and prednisone [CHOP])–like, high-dose methotrexate with asparaginase (Aspa-MTX) chemotherapies, and not otherwise specified (NOS) treatments. Thirty patients received allogeneic hematopoietic cell transplantation (allo-HCT), and 4 patients received autologous hematopoietic cell transplantation. There was no difference in survival between patients receiving AML-like, ALL-like, or Aspa-MTX regimens; survival was longer in patients who received AML-like, ALL-like, or Aspa-MTX regimens than in those who received CHOP-like regimens or NOS. Eleven patients are in persistent complete remission after allo-HCT with a median survival of 49 months vs 8 for other patients. Our series confirms a high response rate with a lower toxicity profile with the Aspa-MTX regimen, offering the best chance of access to hematopoietic cell transplantation and a possible cure.