Fiche publication
Date publication
décembre 2019
Journal
Blood advances
Auteurs
Membres identifiés du Cancéropôle Est :
Pr ADOTEVI Olivier
,
Dr BINDA Delphine
,
Dr CAILLOT Denis
,
Pr DECONINCK Eric
,
Dr FERRAND Christophe
,
Pr GARNACHE-OTTOU Francine
,
Pr MAYNADIE Marc
,
Pr SEILLES Estelle
,
Pr CALLANAN Mary
,
Dr DEBLIQUIS Agathe
,
Dr DRENOU Bernard
,
Dr ANGELOT-DELETTRE Fanny
,
Dr DORVAUX Véronique
Tous les auteurs :
Garnache-Ottou F, Vidal C, Biichlé 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, Geneviève 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, Maynadié 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, Féger 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
Lien Pubmed
Résumé
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.
Référence
Blood Adv. 2019 Dec 23;3(24):4238-4251
