15, 16 CD303 is emerging as another marker useful in the diagnosis of BPDCN and may serve as a potential marker for further directed therapy. 4, 7 TCF4/CD123 coexpression has also been found to be a sensitive and specific diagnostic marker for BPDCN. 4 A diagnosis of BPDCN requires expression of at least 4 of these 6 antigens on malignant cells: CD123 (also referred to as interleukin-3 receptor-alpha ), CD4, CD56, TCL-1, CD2AP, and CD303/BDCA-2, in the absence of lineage-specific markers. 4 This is particularly true when BPDCN presents as isolated cutaneous lesions, because biopsy specimens from cutaneous lesions may not yield sufficient cells for appropriate flow cytometric analysis. 4ĭiagnosis of BPDCN can be difficult due to overlapping morphologic, immunophenotypic, and clinical features of other hematologic malignancies, such as AML. A lumbar puncture is highly recommended at initial diagnosis to rule out CNS disease, and subsequent intrathecal prophylaxis is strongly encouraged even in the absence of known CNS disease. ![]() 4 If extramedullary disease and/or lymphadenopathy is suspected, a PET/CT scan is recommended. It is essential to differentiate the skin lesions of BPDCN from other neoplastic and nonneoplastic skin lesions and rashes, including leukemia cutis associated with acute myeloid leukemia (AML), and analysis by experienced hematopathologists is often required. ![]() Analysis of skin lesions often occurs in collaboration with dermatology. These analyses should include dendritic cell morphology assessment, immunohistochemistry, flow cytometry, cytogenetic analysis (including karyotyping and/or fluorescence in situ hybridization), and molecular analyses. ![]() Analyses of peripheral blasts bone marrow biopsy and aspirate biopsy of skin lesions and, if suspected to be involved, lymph nodes and other tissues are recommended. Laboratory evaluations include a comprehensive metabolic panel and a complete blood cell (CBC) count including platelets and a differential of white blood cells. 8, 13, 14Įvaluation and initial workup for suspected BPDCN consists of a comprehensive medical history and physical examination. 8 Central nervous system (CNS) involvement is not infrequent approximately 10% of patients who present with neurologic symptoms at diagnosis have confirmed CNS involvement, 12 and rates of CNS involvement, both at diagnosis and at relapse, have been found to be in the range of 9% to 26% in several additional studies. 4, 7, 11 Less commonly, patients may present with features of an acute leukemia without skin manifestations. Additional sites of involvement can include lymph nodes, spleen, and other extramedullary organs. 7, 8 Peripheral blood and bone marrow involvement may be minimal at presentation, but tend to develop as the disease progresses. The most frequent clinical presentation of typical BPDCN cases is asymptomatic solitary or multiple skin lesions that can disseminate rapidly without therapy. ![]() 8, 10 Median age of presentation is 65 to 67 years, with an approximate male-to-female ratio of 3:1. 7, 9 BPDCN is associated with a poor prognosis, with a median overall survival (OS) of approximately 8 to 12 months when patients are treated with chemotherapy. 7, 8 The etiology of BPDCN is unknown, but its association with myelodysplastic syndromes or chronic myelomonocytic leukemia in some cases may suggest a related pathogenesis. 6 Pathologically, it is characterized by aggressive proliferation of precursors of PDCs. WHO classification system considers BPDCN to be a subtype of a group of malignancies known as plasmacytoid dendritic cell neoplasms. 3, 4 In 2016, it was recognized as a unique myeloid malignancy. 1, 2 BPDCN, which was formerly known as blastic natural killer cell lymphoma or granular CD4+/CD56+ hematodermic neoplasm, was renamed in the 2008 WHO classification with the evolving knowledge of its PDC origin. Management of Blastic Plasmacytoid Dendritic Cell Neoplasmīlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare myeloid malignancy, representing only 0.44% of hematologic malignancies, with an incidence of 0.04 cases per 100,000 people in the United States.
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