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      CT findings associated with blastic plasmacytoid dendritic cell neoplasm: a case report

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          Abstract

          Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that is frequently misdiagnosed. We present a case of a 53-year-old man diagnosed with blastic plasmacytoid dendritic cell neoplasm with extensive computed tomography (CT) findings and provide an imaging focused review of this uncommon malignancy.

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

          , Francesco Mannelli, Alfonso D'Arco … (2013)
          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.
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            Exome sequencing reveals novel and recurrent mutations with clinical impact in blastic plasmacytoid dendritic cell neoplasm.

            L Brichs, S Montes, G. Gomez … (2014)
            Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare disease that currently lacks genomic and genetic biomarkers to assist in its clinical management. We performed whole-exome sequencing (WES) of three BPDCN cases. Based on these data, we designed a resequencing approach to identify mutations in 38 selected genes in 25 BPDCN samples. WES revealed 37-99 deleterious gene mutations per exome with no common affected genes between patients, but with clear overlap in terms of molecular and disease pathways (hematological and dermatological disease). We identified for the first time deleterious mutations in IKZF3, HOXB9, UBE2G2 and ZEB2 in human leukemia. Target sequencing identified 29 recurring genes, ranging in prevalence from 36% for previously known genes, such as TET2, to 12-16% for newly identified genes, such as IKZF3 or ZEB2. Half of the tumors had mutations affecting either the DNA methylation or chromatin remodeling pathways. The clinical analysis revealed that patients with mutations in DNA methylation pathway had a significantly reduced overall survival (P=0.047). We provide the first mutational profiling of BPDCN. The data support the current WHO classification of the disease as a myeloid disorder and provide a biological rationale for the incorporation of epigenetic therapies for its treatment.
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              Blastic plasmacytoid dendritic cell neoplasm: update on molecular biology, diagnosis, and therapy.

              Ling-Li Zhang, Pedro Horna, Wasif Riaz … (2014)
              Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with an aggressive clinical course. Most patients with BPDCN have skin lesions and simultaneous involvement of the peripheral blood, bone marrow, and lymph nodes. A search of PubMed and Medline was conducted for English-written articles relating to BPDCN, CD4(+)CD56(+) hematodermic neoplasm, and blastic natural killer cell lymphoma. Data regarding diagnosis, prognosis, and treatment were analyzed. BPDCN is derived from precursor plasmacytoid dendritic cells. The diagnosis of BPDCN is based on the characteristic cytology and immunophenotype of malignant cells coexpressing CD4, CD56, CD123, blood dendritic cell antigens 2 and 4, and CD2AP markers. Multiple chromosomal abnormalities and gene mutations previously reported in patients with myeloid and selected lymphoid neoplasms were identified in approximately 60% of patients with BPDCN. Prospectively controlled studies to guide treatment decisions are lacking. The overall response rate with aggressive acute lymphoblastic leukemia-type induction regimens was as high as 90%, but the durability of response was short. Median survival rates ranged between 12 and 16 months. Patients with relapsed disease may respond to L-asparaginase-containing regimens. Allogeneic hematopoietic stem cell transplantation, particularly when performed during the first remission, may produce durable remissions in selected adults. BPDCN is a rare aggressive disease that typically affects elderly patients. The most commonly affected nonhematopoietic organ is the skin. Although BPDCN is initially sensitive to conventional chemotherapy regimens, this response is relatively short and long-term prognosis is poor. In the near future, novel targeted therapies may improve outcomes for patients with BPDCN.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Acta Radiol Open
                Journal ID (iso-abbrev): Acta Radiol Open
                Journal ID (publisher-id): ARR
                Journal ID (hwp): sparr
                Title: Acta Radiologica Open
                Publisher: SAGE Publications (Sage UK: London, England )
                ISSN (Electronic): 2058-4601
                Publication date (Electronic): 26 July 2016
                Publication date Collection: July 2016
                Volume: 5
                Issue: 7
                Electronic Location Identifier: 2058460116657688
                Affiliations
                [1 ]Department of Radiology, Moffitt Cancer Center, Tampa, Florida, USA
                [2 ]Department of Internal Hospitalist Medicine, Moffitt Cancer Center, Tampa, Florida, USA
                [3 ]Department of Malignant Hematology, Moffitt Cancer Center, Tampa, Florida, USA
                Author notes
                [*]Daniel Jeong, Department of Radiology, Moffitt Cancer Center, 12902 USF Magnolia Drive, Tampa, FL 33612, USA. Email: Daniel.jeong@ 123456moffitt.org
                Article
                Publisher ID: 10.1177_2058460116657688
                DOI: 10.1177/2058460116657688
                PMC ID: 4963986
                PubMed ID: 27504192
                SO-VID: d54d3cf4-9e44-4aeb-b87e-cebc9aed6317
                Copyright © © The Foundation Acta Radiologica 2016
                License:

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 3.0 License ( http://www.creativecommons.org/licenses/by-nc/3.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                Date received : 17 December 2015
                Date accepted : 10 June 2016
                Categories
                Subject: Case Report
                Custom metadata
                edited-state corrected-proof

                Keywords: blastic plasmacytoid dendritic cell neoplasm (bpdcn),hematologic malignancy
                Data availability:
                Keywords: blastic plasmacytoid dendritic cell neoplasm (bpdcn), hematologic malignancy

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