Blood advances · 2019

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

СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди

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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, Le Garff-Tavernier Magali, 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

Аннотация

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.

Предметные рубрики MeSH

Acute Disease Biomarkers Blood Cell Count Bone Marrow Chromosome Aberrations Clonal Evolution Dendritic Cells Disease Management Hematopoietic Stem Cell Transplantation Humans Immunophenotyping Leukemia Neoplasm Metastasis Neoplasm Staging Prognosis Treatment Outcome

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

Ошибки и ограничения

Аннотация

Предметные рубрики MeSH