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

Garnache-Ottou, F.; Vidal, C.; Biichle, 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, E. A.; Harrivel, V.; Desbrosses, Y.; Gruson, B.; Genevieve, F.; Thepot, S.; Drebit, Y.; Leguay, T.; Gros, F. -X.; Lechevalier, N.; Saussoy, P.; Salaun, V.; Cornet, E.; Benseddik, Z.; Veyrat-Masson, R.; Wagner-Ballon, O.; Salanoubat, C.; Maynadie, M.; Guy, J.; Caillot, D.; Jacob, M. -C.; Cahn, J. -Y.; Gressin, R.; Rose, J.; Quesnel, B.; Guerin, E.; Trimoreau, F.; Feuillard, J.; Gourin, M. -P.; 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, M. -C.; Peterlin, P.; Ticchioni, M.; Rohrlich, P. -S.; 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.; Feger, 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, A. -C.; Eischen, A.; Mayeur-Rousse, C.; Guffroy, B.; Recher, C.; Loosveld, M.; Garnier, A.; Barlogis, V.; Rosenthal, M. A.; 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.

doi:10.1182/bloodadvances.2019000647

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, CD3031, CD3041, and cTCL11) 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.

How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients? / Garnache-Ottou, F.; Vidal, C.; Biichle, 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, E. A.; Harrivel, V.; Desbrosses, Y.; Gruson, B.; Genevieve, F.; Thepot, S.; Drebit, Y.; Leguay, T.; Gros, F. -X.; Lechevalier, N.; Saussoy, P.; Salaun, V.; Cornet, E.; Benseddik, Z.; Veyrat-Masson, R.; Wagner-Ballon, O.; Salanoubat, C.; Maynadie, M.; Guy, J.; Caillot, D.; Jacob, M. -C.; Cahn, J. -Y.; Gressin, R.; Rose, J.; Quesnel, B.; Guerin, E.; Trimoreau, F.; Feuillard, J.; Gourin, M. -P.; 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, M. -C.; Peterlin, P.; Ticchioni, M.; Rohrlich, P. -S.; 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.; Feger, 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, A. -C.; Eischen, A.; Mayeur-Rousse, C.; Guffroy, B.; Recher, C.; Loosveld, M.; Garnier, A.; Barlogis, V.; Rosenthal, M. A.; 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.. - In: BLOOD ADVANCES. - ISSN 2473-9529. - 3:24(2019), pp. 4238-4251. [10.1182/bloodadvances.2019000647]