Case for diagnosis

Maio, Paula; Fernandes, Cândida; Afonso, Ana; Sachse, Fernanda; Cabeçadas, José; Cardoso, Jorge

doi:10.1590/s0365-05962013000100022

Cited by 4 publications

(3 citation statements)

References 6 publications

(3 reference statements)

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“…However, pediatric cases, even in infants and young children, have also been reported (14,15). Clinically, there is nearly always multiple organ involvement, and cutaneous involvement characterized with tumor cells located under the dermis is the main initial presentation of BPDCN in the majority of the cases, although occasionally there is no cutaneous involvement and the disease presents with leukemia symptoms (16,17); in addition, extracutaneous involvement often occurs simultaneously, such as in the lymph nodes, peripheral blood, bone marrow, skeleton, gastrointestinal tract, central nervous system, lung, mediastinum and pancreas (18)(19)(20)(21); however, to the best of our knowledge, testicular involvement has not been reported to date. The patient in case 1 exhibited typical clinical manifestations, with initial skin involvement, followed by rapid occurrence of multiple organ involvement (lymph nodes, liver, spleen and bone marrow); as regards the second patient, there was simultaneous occurrence of bilateral maxillofacial involvement, confirmed by histopathological examination of a surgical biopsy specimen, bilateral testicular involvement and mediastinal involvement, confirmed by histopathological examination of a needle biopsy specimen, prior to bone marrow involvement.…”

Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm in children: A review of two cases

Deng

Yang

Kuang

et al. 2017

Molecular and Clinical Oncology

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Abstract. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly characterized, rare malignant tumor of the skin and hematopoietic system. BPDCN occurs mainly in the elderly, whereas it is rarer among children, and has variable clinical manifestations. Optimal chemotherapeutic regimens for the treatment of BPDCN have not yet been determined and this tumor has a poor prognosis. In this study, two pediatric cases of BPDCN, including a 7-year-old female and a 9-year-old male patient, diagnosed at the Xiangya Hospital of Central South University over the past 2 years, were retrospectively reviewed. Both cases exhibited multiple organ involvement, although the clinical manifestations differed; they were diagnosed with BPDCN based on the clinical manifestations, pathological and immunohistochemical findings, which included positivity for CD4, CD56 and CD123. A high-risk acute lymphocytic leukemia (ALL) chemotherapy regimen was administered to both patients. The patient in the first case achieved a complete remission, but unfortunately her parents refused follow-up treatment and she succumbed to the disease 9 months after the initial diagnosis. The second patient was treated for a total of three courses with a chemotherapy regimen including daunorubicin, cytarabine and etoposide, followed by two courses of the high-risk ALL chemotherapy regimen; unfortunately, a remission was not achieved and the patient was scheduled to receive hematopoietic stem cell transplantation. Thus, not all pediatric BPDCN patients may be able to achieve complete remission following chemotherapy with the high-risk ALL regimen, and other treatment options must be investigated in the future.

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Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm in children: A review of two cases

Deng

Yang

Kuang

et al. 2017

Molecular and Clinical Oncology

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“…The plaque diameter ranges from a few millimeters to over ten centimeters, while the color ranges from dark red to characteristic purple, and ulcers occasionally occur. The manifestations of this disease also occasionally involve the mucosae ( 9 , 10 ). In addition to the initial manifestation of skin lesions, the disease involves other systems.…”

Section: Introductionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm: A case report

Wang

Jia

et al. 2014

Oncology Letters

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Abstract. Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as agranular cluster of differentiation (CD)4 + /CD56 + hematodermic neoplasm, is a rare and aggressive type of lymphoma, with only ~100 cases reported worldwide. BPDCN is a hematological malignancy derived from precursors of plasmacytoid dendritic cells and is clinically characterized by cutaneous manifestations involving the lymph nodes and peripheral blood, a leukemia-like dissemination and a poor prognosis. The present study reports the case of a 54-year-old male who presented with symptoms characteristic of BPDCN. Pathological and immunohistochemical analysis of abdominal skin lesion biopsies were used to determine a diagnosis of stage ⅢE BPDCN. Although cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy was administered, the patient succumbed to BPDCN nine days after the discontinuation of chemotherapy. Thus, the period from BPDCN presentation to mortality was ≤3 months. The case reported in the present study was characterized by rapid development and poor prognosis, and displayed additional features of BPDCN, including systemic dissemination and a short survival period. IntroductionBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly invasive type of malignant hematopoietic and lymphoid tissue tumor (1). Since BPDCN was initially reported by Adachi et al (2) in 1994, it has been successively reported in the literature. In 2004, Chaperot et al (3) identified that the BPDCN tumor functions similarly to plasmacytoid dendritic cells (pDC), and, thus, proposed that it may be derived from the precursor of the pDC. Subsequent studies determined that the BPDCN tumor cells express the highly specific pDC markers, blood dendritic cell antigen (BDCA)-2/cluster of differentiation (CD)303 and BDCA-4/CD304, supporting the hypothesis that the BPDCN tumor is derived from pDC (4). In 2005, the World Health Organisation (WHO) European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas recommended the use of the term CD4 + /CD56 + hematodermic neoplasm (5,6). In 2008, the tumor was officially named BPDCN in the WHO classification of lymphoid and hematopoietic tumors and was listed as a novel, independent type of hematopoietic and lymphoid tissue disease (7).BPDCN can occur in individuals of all ages (range, 8 months-103 years), however, it predominantly occurs in the elderly (8). Skin involvement is the most prominent clinical feature and includes isolated, confined or generalized plaques or nodules. The plaque diameter ranges from a few millimeters to over ten centimeters, while the color ranges from dark red to characteristic purple, and ulcers occasionally occur. The manifestations of this disease also occasionally involve the mucosae (9,10). In addition to the initial manifestation of skin lesions, the disease involves other systems. For example, lymphadenectasis occurs in 40-50% of patients, the bone marrow and peripheral blood are involved in 60-90% of patient...

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“…In the January/February 2013 edition, Maio et al 1 describe a patient with CD4 + /CD56 + /CD123 + ascribed as blastic plasmacytoid dendritic cell neoplasia (BDCN). However, since CD4 + /CD56 + /CD123 + neoplasms are highly heterogeneous, the precise diagnosis requires an extensive immunophenotypic panel.…”

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confidence: 99%