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