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Blastic plasmacytoid dendritic cell neoplasm: diagnostic criteria and therapeutical approaches
Author(s) -
Pagano Livio,
Valentini Caterina G.,
Grammatico Sara,
Pulsoni Alessandro
Publication year - 2016
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.14146
Subject(s) - medicine , plasmacytoid dendritic cell , chemotherapy , interleukin 3 receptor , myeloid , induction chemotherapy , bone marrow , oncology , haematopoiesis , immunology , stem cell , dendritic cell , antigen , biology , genetics
Summary Blastic plasmacytoid dendritic cell neoplasm ( BPDCN ) is a rare haematological malignancy derived from the precursors of plamacytoid dendritic cells, with an aggressive clinical course and high frequency of cutaneous and bone marrow involvement. Neoplastic cells express CD 4, CD 43 (also termed SPN ), CD 45 RA and CD 56 (also termed NCAM 1), as well as the plasmacytoid dendritic cell‐associated antigens CD 123 (also termed IL 3 RA ), BDCA ‐2 (also termed CD 303, CLEC 4E) TCL 1 and CTLA 1 (also termed GZMB ). The median survival is only a few months as the tumour exhibits a progressive course despite initial response to chemotherapy. The best modality of treatment remains to be defined. Generally, patients receive acute leukaemia‐like induction, according to acute myeloid leukaemia ( AML )‐type or acute lymphoid leukaemia ( ALL )‐type regimens. The frequent neuromeningeal involvement indicates systematic pre‐emptive intrathecal chemotherapy in addition to intensive chemotherapy. Allogeneic haematopoietic stem cell transplantation ( HSCT ), particularly when performed in first remission, may improve the survival. Preliminary data suggest a potential role for immunomodulatory agents and novel targeted drugs. Herein epidemiology, clinical manifestations, diagnosis and management of BPDCN will be presented. In detail, this review focuses on the therapeutic aspects of BPDCN , proposing a treatment algorithm for the management of the disease, including induction chemotherapy, allogeneic HSCT and intrathecal prophylaxis at different steps of treatment, according to compliance, biological and clinical characteristics of patients.