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

Francine Garnache-Ottou 1 Chrystelle Vidal 2 Sabeha Biichlé 1 Florian Renosi 1 Eve Poret 1 Maïder Pagadoy 2 Maxime Desmarets 2 Anne Roggy 2 Estelle Seilles 2 Lou Soret 2 Françoise Schillinger Sandrine Puyraimond Tony Petrella Claude Preudhomme Christophe Roumier Elisabeth A. Macintyre 3 Véronique Harrivel Yohan Desbrosses Bérengère Gruson Franck Geneviève Sylvain Thepot 4 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 5 Christine Arnoulet 6 Blandine Benet Véronique Dorvaux Caroline Bret 7, 8 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 9, 10 Mikael Roussel 9, 10 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 11 Marie Loosveld Alice Garnier Vincent Barlogis Maria Alessandra Rosenthal Sophie Brun Nathalie Contentin Sébastien Maury Mary Callanan 12, 13 Christine Lefebvre 13 Natacha Maillard Patricia Okamba Christophe Ferrand 1 Olivier Adotevi 1 Philippe Saas 1 Fanny Angelot-Delettre 1 Delphine Binda 1 Eric Deconinck 1
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.
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Submitted on : Friday, July 10, 2020 - 2:19:14 PM
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Francine Garnache-Ottou, Chrystelle Vidal, Sabeha Biichlé, Florian Renosi, Eve Poret, et al.. How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients?. Blood Advances, The American Society of Hematology, 2019, 3 (24), pp.4238-4251. ⟨10.1182/bloodadvances.2019000647⟩. ⟨hal-02442440⟩

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