Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

被引：259

作者：

Pagano, Livio ^{[1
]}

Valentini, Caterina Giovanna ^{[1
]}

Pulsoni, Alessandro ^{[2
]}

Fisogni, Simona ^{[3
]}

Carluccio, Paola ^{[4
]}

Mannelli, Francesco ^{[5
]}

Lunghi, Monia ^{[6
]}

Pica, Gianmatteo ^{[7
]}

Onida, Francesco ^{[8
]}

Cattaneo, Chiara ^{[9
]}

Piccaluga, Pier Paolo ^{[10
]}

Di Bona, Eros ^{[11
]}

Todisco, Elisabetta ^{[12
]}

Musto, Pellegrino ^{[13
]}

Spadea, Antonio ^{[14
]}

D'Arco, Alfonso ^{[15
]}

Pileri, Stefano ^{[10
]}

Leone, Giuseppe ^{[1
]}

Amadori, Sergio ^{[16
]}

Facchetti, Fabio ^{[3
]}

机构：

[1] Univ Cattolica Sacro Cuore, Inst Hematol, I-00168 Rome, Italy

[2] Univ Roma La Sapienza, Inst Hematol, Rome, Italy

[3] Univ Brescia, Dept Pathol, I-25121 Brescia, Italy

[4] Univ Bari, Div Hematol, I-70121 Bari, Italy

[5] Univ Florence, Dept Hematol, I-50121 Florence, Italy

[6] Amedeo Avogadro Univ Eastern Piedmont, Div Hematol, Novara, Italy

[7] Hosp San Martino Genova, Dept Hematol, Genoa, Italy

[8] Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, UO Ematol CTMO 1, I-20122 Milan, Italy

[9] Spedali Civil Brescia, Dept Hematol, I-25125 Brescia, Italy

[10] Univ Bologna, Dept Hematol & Oncol Sci L&A Seragnoli, I-40126 Bologna, Italy

[11] San Bortolo Hosp, Dept Cellular Therapy & Haematol, Vicenza, Italy

[12] Humanitas Canc Ctr IRCCS, Hematol Unit, Milan, Italy

[13] IRCCS CROB, Div Hematol, Rionero In Vulture, Italy

[14] Regina Elena Inst Canc Res, Div Hematol, Rome, Italy

[15] Nocera Inferiore, Divis Hematol, Rome, Italy

[16] Tor Vergata Univ Hosp, Dept Hematol, Rome, Italy

来源：

HAEMATOLOGICA | 2013年 / 98卷 / 02期

关键词：

HEMATODERMIC NEOPLASM; CUTANEOUS INVOLVEMENT; TRANSPLANTATION; FEATURES; CRITERIA; LINEAGE; CD4(+); SERIES; TUMOR;

D O I：

10.3324/haematol.2012.072645

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The objective of this study was to evaluate the clinical features, prognostic factors, and efficacy of treatments in patients with blastic plasmacytoid dendritic cell neoplasm with a leukemic presentation at onset of the disease. In order to do this, a retrospective multicenter study was performed from 2005-2011 in 28 Italian hematology divisions in which 43 cases were collected. Forty-one patients received an induction therapy, consisting of an acute myeloid leukemia-type regimen in 26 patients (60%) and acute lymphoid leukemia/lymphoma-type regimen in 15 patients (35%). Six patients (14%) underwent allogeneic hematopoietic stem cell transplantation. Seventeen patients (41%) achieved a complete remission: seven after acute myeloid leukemia-type treatment and 10 after an acute lymphoid leukemia/lymphoma-type regimen, with a significant advantage for acute lymphoid leukemia/lymphoma-type chemotherapy (P=0.02). Relapse occurred in six of the 17 patients (35%) who achieved complete remission, more frequently after acute lymphoid leukemia/lymphoma-type chemotherapy. The median overall survival was 8.7 months (range, 0.2-32.9). The patients treated with an acute myeloid leukemia-type regimen had an overall survival of 7.1 months (range, 0.2-19.5), whereas that of the patients receiving acute lymphoid leukemia/lymphoma-type chemotherapy was 12.3 months (range, 1-32.9) (P=0.02). The median overall survival of the allogeneic hematopoietic stem cell transplant recipients was 22.7 months (range, 12-32.9), and these patients had a significant survival advantage compared to the non-transplanted patients (median 7.1 months, 0.2-21.3; P=0.03). In conclusion, blastic plasmacytoid dendritic cell neoplasm with bone-marrow involvement is an aggressive subtype of high-risk acute leukemia. The rarity of this disease does not enable prospective clinical trials to identify the better therapeutic strategy, which, at present, is based on clinicians' experience.

引用

页码：239 / 246

页数：8

共 37 条

[1]

[Anonymous], 2009, HEMATOL MEET REP

[2]

Bueno C, 2004, HAEMATOLOGICA, V89, P58

[3] Identification of a leukemic counterpart of the plasmacytoid dendritic cells [J].

Chaperot, L ;

Bendriss, N ;

Manches, O ;

Gressin, R ;

Maynadie, M ;

Trimoreau, F ;

Orfeuvre, H ;

Corront, B ;

Feuillard, J ;

Sotto, JJ ;

Bensa, JC ;

Brière, F ;

Plumas, J ;

Jacob, MC .

BLOOD, 2001, 97 (10) :3210-3217

[4] Blastic Plasmacytoid Dendritic Cell Neoplasm [J].

Chen, Jieping ;

Zhou, Jingying ;

Qin, Dabing ;

Xu, Shuangnian ;

Yan, Xiaochu .

JOURNAL OF CLINICAL ONCOLOGY, 2011, 29 (02) :E27-E29

[5] Revised recommendations of the international working group for diagnosis, standardization of response criteria, treatment outcomes, and reporting standards for therapeutic trials in acute myeloid leukemia [J].

Cheson, BD ;

Bennett, JM ;

Kopecky, KJ ;

Büchner, T ;

Willman, CL ;

Estey, EH ;

Schiffer, CA ;

Döhner, H ;

Tallman, MS ;

Lister, TA ;

LoCocco, F ;

Willemze, R ;

Biondi, A ;

Hiddemann, W ;

Larson, RA ;

Löwenberg, B ;

Sanz, MA ;

Head, DR ;

Ohno, R ;

Bloomfield, CD .

JOURNAL OF CLINICAL ONCOLOGY, 2003, 21 (24) :4642-4649

[6] Cutaneous Manifestations of Blastic Plasmacytoid Dendritic Cell Neoplasm-Morphologic and Phenotypic Variability in a Series of 33 Patients [J].

Cota, Carlo ;

Vale, Esmeralda ;

Viana, Isabel ;

Requena, Luis ;

Ferrara, Gerardo ;

Anemona, Lucia ;

Metze, Dieter ;

Fink-Puches, Regina ;

Wiesner, Thomas ;

Cerroni, Lorenzo .

AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 2010, 34 (01) :75-87

[7] Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? [J].

Dalle, S. ;

Beylot-Barry, M. ;

Bagot, M. ;

Lipsker, D. ;

Machet, L. ;

Joly, P. ;

Dompmartin, A. ;

d'Incan, M. ;

Maubec, E. ;

Grange, F. ;

Dereure, O. ;

Prey, S. ;

Barete, S. ;

Wetterwald, M. ;

Fraitag, S. ;

Petrella, T. .

BRITISH JOURNAL OF DERMATOLOGY, 2010, 162 (01) :74-79

[8] Blastic Plasmacytoid Dendritic Cell Neoplasia (BPDC) in Elderly Patients: Results of a Treatment Algorithm Employing Allogeneic Stem Cell Transplantation with Moderately Reduced Conditioning Intensity [J].

Dietrich, Sascha ;

Andrulis, Mindaugas ;

Hegenbart, Ute ;

Schmitt, Thomas ;

Bellos, Frauke ;

Martens, Uwe M. ;

Meissner, Julia ;

Kraemer, Alwin ;

Ho, Anthony D. ;

Dreger, Peter .

BIOLOGY OF BLOOD AND MARROW TRANSPLANTATION, 2011, 17 (08) :1250-1254

[9] Gene-expression profiling and array-based CGH classify CD4+CD56+ hematodermic neoplasm and cutaneous myelomonocytic leukemia as distinct disease entities [J].

Dijkman, Remco ;

van Doorn, Remco ;

Szuhai, Karoly ;

Willemze, Rein ;

Vermeer, Maarten H. ;

Tensen, Cornelis P. .

BLOOD, 2007, 109 (04) :1720-1727

[10]

Facchetti F., 2008, WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues, V4th, P145

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