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

被引：260

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

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