Aims: To evaluate the clinical features, the prognostic factors, and the efficacy of treatments in patients (pts) with Blastic Plasmacytoid Dendritic Cell Leukemia (BPDCL) collected among GIMEMA centers. Methods: A retrospective multicenter study was carried out between January 2005 and December 2010 in 28 Italian hematology divisions. Results: According to WHO2008 classification 49 evaluable cases of BPDCL were collected (M/F 38/11; median age 51 yo, range 20-81). At diagnosis the median bone marrow infiltration was 90% with a poor residual bone marrow function, as documented by low hemoglobin level (median value 6.2 g/dL) and platelet count (median count 23x10^9/L); 42 pts (86%) had peculiar skin lesions, while lymph nodes and/or spleen involvements were documented in 25 cases (51%), and extramedullary disease in 8 (16%). In 29 pts (59%) cytogenetic study was performed, revealing an unfavourable karyotype in 11. Forty-five pts received an acute leukemia-like induction therapy (4 died early), consisting of antracycline/cytarabine (AML-like regimen) in 33 (73%), and of dexamethasone/vincristine/cyclophosphamide/metotrexate (ALL-like regimen) in 12 (27%); overall 9 pts (20%) underwent allo-HSCT. Complete (CR) or partial remission (PR) after induction therapy was achieved in 21 and 6 pts respectively (overall response rate 60%). It were registered 12 CR and 4 PR after AML-like regimen, and 9 CR and 2 PR after ALL-like regimen, with a significant advantage for ALL-like chemotherapy (p=0.01). The median overall survival (OS) was 8 months (range 0.2-60); 6 months (range 0.2-60) and 12 months (range 1.8-31) in pts received AML-like regimen and ALL-like regimen respectively (p=0.06). In HSCT-pts the median OS was 31 months (range 3-60), with a significant advantage with respect to non-transplanted pts (median 6 months, range 0.2-26, p=0.002). In pts obtaining a complete remission, the median DFS was 9 months (range 3-60); among them 18 relapsed, after a median time to diagnosis of 4 months (range 1-9). Conclusions: BPDCL is a rare but clinically aggressive hematopoietic neoplasia, preferentially involving skin and bone marrow and behaving similar to high-risk acute leukemia. Initial response to ALL-like induction chemotherapy is good, but relapse occurred rapidly after a median time of only 4 months; allo-HSCT performed in first remission may lead to long-term survival and disease control in selected cases, but more data are needed to confirm these results.

Blastic plasmacytoid dendritic cell leukemia: preliminary results of a retrospective italian multicentric study.

BONIFACIO, Massimiliano;
2011

Abstract

Aims: To evaluate the clinical features, the prognostic factors, and the efficacy of treatments in patients (pts) with Blastic Plasmacytoid Dendritic Cell Leukemia (BPDCL) collected among GIMEMA centers. Methods: A retrospective multicenter study was carried out between January 2005 and December 2010 in 28 Italian hematology divisions. Results: According to WHO2008 classification 49 evaluable cases of BPDCL were collected (M/F 38/11; median age 51 yo, range 20-81). At diagnosis the median bone marrow infiltration was 90% with a poor residual bone marrow function, as documented by low hemoglobin level (median value 6.2 g/dL) and platelet count (median count 23x10^9/L); 42 pts (86%) had peculiar skin lesions, while lymph nodes and/or spleen involvements were documented in 25 cases (51%), and extramedullary disease in 8 (16%). In 29 pts (59%) cytogenetic study was performed, revealing an unfavourable karyotype in 11. Forty-five pts received an acute leukemia-like induction therapy (4 died early), consisting of antracycline/cytarabine (AML-like regimen) in 33 (73%), and of dexamethasone/vincristine/cyclophosphamide/metotrexate (ALL-like regimen) in 12 (27%); overall 9 pts (20%) underwent allo-HSCT. Complete (CR) or partial remission (PR) after induction therapy was achieved in 21 and 6 pts respectively (overall response rate 60%). It were registered 12 CR and 4 PR after AML-like regimen, and 9 CR and 2 PR after ALL-like regimen, with a significant advantage for ALL-like chemotherapy (p=0.01). The median overall survival (OS) was 8 months (range 0.2-60); 6 months (range 0.2-60) and 12 months (range 1.8-31) in pts received AML-like regimen and ALL-like regimen respectively (p=0.06). In HSCT-pts the median OS was 31 months (range 3-60), with a significant advantage with respect to non-transplanted pts (median 6 months, range 0.2-26, p=0.002). In pts obtaining a complete remission, the median DFS was 9 months (range 3-60); among them 18 relapsed, after a median time to diagnosis of 4 months (range 1-9). Conclusions: BPDCL is a rare but clinically aggressive hematopoietic neoplasia, preferentially involving skin and bone marrow and behaving similar to high-risk acute leukemia. Initial response to ALL-like induction chemotherapy is good, but relapse occurred rapidly after a median time of only 4 months; allo-HSCT performed in first remission may lead to long-term survival and disease control in selected cases, but more data are needed to confirm these results.
blastic plasmacytoid dendritic leukemia; rare disease; chemotherapy
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11562/893597
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