CD4+/CD56+ Hematodermic Neoplasm

Piña‐Oviedo, Sergio; Herrera-Medina, H; Coronado, H; Valle, Luis Del; Ortíz-Hidalgo, Carlos

doi:10.1097/01.pai.0000213139.39654.40

Cited by 7 publications

(3 citation statements)

References 42 publications

(55 reference statements)

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“…124,127,134-136 Expression of CD3 should raise suspicion for a T-lineage malignancy, although there have been rare cases of BPDCN reported with cytoplasmic CD3 expression. 111,137 Furthermore, some cases may show expression of S-100 (zonal distribution; Table 3), 138,139 CD10, 135 or CD117. 135 cD 34, CD8, myeloperoxidase, lysozyme, PAX5, CD20, CD79a, Epstein Barr-Virus, and T-cell receptor protein are consistently negative, and expression of such markers should exclude the diagnosis of BPDCN.…”

Section: Blastic Plasmacytoid Dendritic Cell Neoplasmmentioning

confidence: 99%

Plasmacytoid Dendritic Cells

Jegalian

Facchetti²,

Jaffe³

2009

Advances in Anatomic Pathology

160

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Plasmacytoid dendritic cells (PDCs) have perplexed pathologists for decades, undergoing multiple adjustments in nomenclature as their lineage and functions have been characterized. Although PDCs account for less than 0.1% of peripheral blood mononuclear cells, they serve as a principal source of interferon-alpha and are also known as interferon-I producing cells (IPCs). Upon activation in vitro, they can differentiate into dendritic cells, and recent studies have substantiated a potential role in antigen presentation. Thus, PDCs may act as a link between innate and adaptive immunity. Normally found in small quantities in primary and secondary lymphoid organs, PDCs accumulate in a variety of inflammatory conditions, including Kikuchi-Fujimoto lymphadenopathy, hyaline-vascular Castleman disease, and autoimmune diseases, and in certain malignancies such as classical Hodgkin lymphoma and carcinomas. Demonstrating potential for neoplastic transformation reflective of varying stages of maturation, clonal proliferations range from PDC nodules most commonly associated with chronic myelomonocytic leukemia to the rare but highly aggressive malignancy now known as blastic plasmacytoid dendritic cell neoplasm (BPDCN). Formerly called blastic natural killer cell lymphoma or CD4/CD56 hematodermic neoplasm, BPDCN, unlike natural killer cell lymphomas, is not associated with Epstein-Barr virus infection and is generally not curable with treatment regimens for non-Hodgkin lymphomas. In fact, this entity is no longer considered to be a lymphoma and instead represents a unique precursor hematopoietic neoplasm. Acute leukemia therapy regimens may lead to sustained clinical remission of BPDCN, with bone marrow transplantation in first complete remission potentially curative in adult patients.

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Section: Blastic Plasmacytoid Dendritic Cell Neoplasmmentioning

confidence: 99%

Plasmacytoid Dendritic Cells

Jegalian

Facchetti²,

Jaffe³

2009

Advances in Anatomic Pathology

160

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“…The diagnosis of BPDCN may be by immunophenotyping of blastic cells by flow cytometry or immunohistochemistry, because the clonal cells co-express CD4, CD56, and CD123 (dendritic cell-associated antigen) [4,5]. …”

Section: Introductionmentioning

confidence: 99%

Blastic Plasmacytoid Dendritic Cell Neoplasm with Pulmonary Involvement and Atypical Skin Lesion

et al. 2017

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Patient: Female, 51Final Diagnosis: Blastic plasmacytoid dendritic cell neoplasmSymptoms: Pulmonary bleeding • small skin lesionMedication: Hyper-CVAD • methotrexate • cytarabineClinical Procedure: —Specialty: HematologyObjective:Rare diseaseBackground:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematodermic malignancy neoplasm with highly aggressive course and poor prognosis. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood.Case Report:Here, we report the case of a 51-year-old woman who presented a violaceus skin lesion on the lateral region of the right thigh, weight loss, fever, and lymphadenopathies. Computed tomography (CT) displayed thoracic and abdominal lymph node and alveolar bleeding. Flow cytometry from circulating blastic cells was compatible with BPDCN (CD4+, CD56+ and CD123+). She underwent 5 cycles of hyper-CVAD alternating with high-dose methotrexate and cytarabine, but the patient died due to alveolar bleeding and sepsis.Conclusions:We report a rare case of BPDCN characterized by an aggressive course, presence of atypical skin lesion, a finding suggestive of pulmonary infiltration, and nonresponse to induction chemotherapy, leading to late diagnosis and therapeutic management. Because of the late recognition of the skin lesion, neoplastic cells infiltrated the dermis and spread as the disease progressed rapidly to a fatal course.

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“…The diagnosis of BPDCN based on the results of immunohistochemical analysis requires positivity for CD4 and CD56, along with other markers that are more restricted to plasmacytoid dendritic cells (such as CD123), and negativity for lymphoid, NK and myeloid lineage-associated antigens (8,9). BPDCN is typically identified in the CD45 dim+ flow cytometry blast gate region; 'dim' indicates weakly positive detection.…”

Section: Case Reportmentioning

confidence: 99%