Diagnostic and therapeutic advances in blastic plasmacytoid dendritic cell neoplasm: a focus on hematopoietic cell transplantation. Biol Blood Marrow Transplant 2013 Jul;19(7):1006-12
Date
02/12/2013Pubmed ID
23396213DOI
10.1016/j.bbmt.2013.01.027Scopus ID
2-s2.0-84879409907 (requires institutional sign-in at Scopus site) 74 CitationsAbstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an exceedingly rare disorder categorized under acute myeloid leukemia by the World Health Organization. Phenotypically, malignant cells coexpress CD4(+) and CD56(+) without coexpressing common lymphoid or myeloid lineage markers. BPDCN frequently expresses CD123, TCL1, BDCA-2, and CD2AP. Restriction of CD2AP expression to plasmacytoid dendritic cells makes it a useful tool to help confirm diagnosis. Clonal complex chromosome aberrations are described in two-thirds of cases. Eighty percent of BPDCN cases present with nonspecific dermatological manifestations, prompting inclusion in the differential diagnosis of atypical skin rashes refractory to standard treatment. Prognosis is poor, with a median survival of less than 18 months. No prospective randomized data exist to define the most optimal frontline chemotherapy. Current practice considers acute myeloid leukemia-like or acute lymphoblastic leukemia-like regimens acceptable for induction treatment. Unfortunately, responses are short-lived, with second remissions difficult to achieve, underscoring the need to consider hematopoietic cell transplantation early in the disease course. Allografting, especially if offered in first remission, can result in long-term remissions. Preclinical data suggest a potential role for immunomodulatory agents in BPCDN. However, further research efforts are needed to better understand BPDCN biology and to establish evidence-based treatment algorithms that might ultimately improve overall prognosis of this disease.
Author List
Kharfan-Dabaja MA, Lazarus HM, Nishihori T, Mahfouz RA, Hamadani MAuthor
Mehdi H. Hamadani MD Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Adaptor Proteins, Signal TransducingAntigens, CD
Biomarkers, Tumor
Cytoskeletal Proteins
Dendritic Cells
Exanthema
Hematopoietic Stem Cell Transplantation
Humans
Immunologic Factors
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Prognosis
Recurrence
Remission Induction
Survival Analysis
Transplantation, Homologous
