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

被引：253

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
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
[J]. BLOOD, 2001, 97 (10) : 3210 - 3217
[4] Blastic Plasmacytoid Dendritic Cell Neoplasm
Chen, Jieping
Zhou, Jingying
Qin, Dabing
Xu, Shuangnian
Yan, Xiaochu
[J]. 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
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
[J]. 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
Cota, Carlo
Vale, Esmeralda
Viana, Isabel
Requena, Luis
Ferrara, Gerardo
Anemona, Lucia
Metze, Dieter
Fink-Puches, Regina
Wiesner, Thomas
Cerroni, Lorenzo
[J]. AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 2010, 34 (01) : 75 - 87
[7] Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice?
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.
[J]. 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
Dietrich, Sascha
Andrulis, Mindaugas
Hegenbart, Ute
Schmitt, Thomas
Bellos, Frauke
Martens, Uwe M.
Meissner, Julia
Kraemer, Alwin
Ho, Anthony D.
Dreger, Peter
[J]. 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
Dijkman, Remco
van Doorn, Remco
Szuhai, Karoly
Willemze, Rein
Vermeer, Maarten H.
Tensen, Cornelis P.
[J]. 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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