Blastic Plasmacytoid Dendritic Cell Neoplasm: Update on Molecular Biology, Diagnosis, and Therapy (original) (raw)
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Case Reports in Transplantation, 2011
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare and aggressive malignancy that usually presents with diffuse cutaneous lesions. While a favorable response to therapy occurs in a majority of cases, a sustained long-term response is uncommon. Most patients subsequently relapse within a year. In the following report, we present the case of a 41-year-old woman who has not displayed many of the clinical features traditionally associated with BPDCN. The patient received sporadic chemotherapy treatment over the course of 2 years, before undergoing an allogeneic stem cell transplant. Although she ultimately relapsed following her transplant, her disease has repeatedly returned into remission after donor lymphocyte infusion (DLI). Currently, the patient is in remission following her fourth DLI. We believe that allogeneic transplantation should be considered as front-line therapy for the treatment of this rare malignancy.
Acta Haematologica Polonica, 2020
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare aggressive hematopoietic malignancy. The median survival is approximately 12 months, and for patients >65 years the survival rate is 7 months, when only chemotherapy is administered. Clinically, it is characterized by skin involvement and most often bone marrow lesions accompanied by lymphadenopathy and in some cases hepato- and/or splenomegaly. The diagnosis is based on histopathological examination of the skin or bone marrow lesions and tumor cell immunophenotyping. The etiopathogenesis of the disease is not fully understood. Therapeutic decisions are based only on the results of a few retrospective analyses and case reports. This article presents the important role of allogeneic hematopoietic cell transplantation in the treatment of BPDCN.
Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice?
British Journal of Dermatology, 2010
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) derived from precursors of plasmacytoid dendritic cells is a very rare, unique, and highly aggressive immature hematopoietic malignancy, more frequently occurring among healthy elderly adults. BPDCN can be characterized by a striking predilection for cutaneous involvement, which is often detected incidentally by dermatologists and is difficult to clinically distinguish it from other primary skin lesions and histologically from leukemia/lymphoma cutis. Thus, histological diagnosis of cutaneous biopsies is crucial to correctly classify this entity. Most patients eventually progress to acute myeloid leukemia and are generally not curable. Here, we present 2 cases of classic BPDCN and discuss the origin of tumor and literature-based characteristic clinical and morphological features, evolving immunomarkers, and molecular genetic aspects of this neoplasm.
Excessive therapeutic response in a case of blastic plasmacytoid dendritic cell neoplasm
Clinical advances in hematology & oncology : H&O, 2012
A 65-year-old African American man had been followed in the hematology-oncology clinic since 2003 for monoclonal gammopathy of unknown significance (MGUS) and anemia. Bone marrow biopsy at the time of initial presentation was unremarkable. In August 2008, he began to develop violaceous cutaneous lesions, generalized lymphadenopathy, and pancytopenia. Punch biopsy of the left arm revealed superficial and deep perivascular and interstitial infiltrates of small-to intermediate-sized cells. A bone marrow biopsy in October 2008 showed infiltration of mononuclear cells of similar morphology and immunohistochemical phenotype (positive for CD4 and CD56). Additionally, flow cytometry of a cervical lymph node biopsy from the same month demonstrated a large population of cells expressing CD4, CD56, and CD123. Based on these findings, the diagnosis of blastic plasmacytoid dendritic cell neoplasm, formerly known as CD4-positive/CD56-positive hematodermic neoplasm or blastic natural killer cell lymphoma, was made. From November 2008 to June 2009, the patient received 8 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy, with a good response. His cutaneous lesions and palpable lymph nodes rapidly decreased in size after the third cycle. Positron emission tomography (PET)/computed tomography (CT) scans indicated complete response after the eighth cycle. However, in August 2009, while evaluation for stem cell transplantation was being arranged, he again developed similar extensive cutaneous lesions over his
The advances in therapy of blastic plasmacytoid dendritic cell neoplasm
Expert opinion on investigational drugs, 2018
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive myeloid malignancy that contributes to <1% of all hematologic neoplasms. Before the introduction of various targeted agents, the therapeutic approach was based on regimens used for acute lymphoblastic or myeloid leukemia and non-Hodgkin's lymphoma (e.g. hyperCVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin and dexamethasone alternating with high dose methotrexate and cytarabine) and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) -based regimens) followed by allogeneic stem cell transplantation for eligible patients. Given that this disease primarily affects older patients, there is a significant barrier to using these highly toxic regimens, even though these regimens are usually associated with the most durable response Areas covered: In this review, we briefly discuss outcomes with the use of leukemia-based induction regimens as well as the use of stem cell transpla...
Dendritic Cell Leukemia: a Review
Current Oncology Reports, 2020
Purpose of Review The purpose of this review was to summarize the clinical, diagnostic, and therapeutic features of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Recent Findings Several case reports and series revealed new clinical, molecular, diagnostic, and therapeutic aspects of the disease. The clinical presentation diversity has been confirmed, with frequent leukemic non-cutaneous or rare atypical manifestations. The clonal evolution in the development of BPDCN has not been sufficiently elucidated. Although certain immunophenotypic markers (CD4, TCL1, CD123, CD56, CD303) are indicative of BPDCN, the diagnosis remains in certain cases challenging. Adult (ALL)-type chemotherapy followed by hematopoietic stem cell transplantation (HSCT) is related to a favorable outcome, while chemotherapy alone seems enough in children. Future studies should continue to investigate whether CD123-directed therapies could be utilized. Summary BPDCN is a rare aggressive malignancy that needs an aggressive induction therapy. Although a diagnostic consensus is still lacking, and large retrospective studies are also needed to obtain standardized treatment guidelines, the future perspectives are encouraging, because of novel therapeutic agents that are under investigation.
A Rare Case Report of Blastic Plasmacytoid Dendritic Cell Neoplasm
American Journal of Medical Case Reports, 2020
Blastic plasmacytoid dendritic cell neoplasm also formerly known as Blastic NK cell lymphoma is a very rare haematological malignancy. WHO defined BPDCN as a neoplasm with features of cutaneous lymphoma and/or leukaemia and designated BPDCN in a separate category under the myeloid class of neoplasms since 2008. Diagnosis of BPDCN requires both morphological evidence of plasmacytoid dendritic blast cells and immunohistochemical positivity for CD123, CD4, CD56 and TLC-1. Unlike other haematological malignancies there are no established optimal chemotherapy regimens for BPCDN although standard chemotherapeutic regimens used for induction treatment of AML, ALL and high grade lymphoma have shown complete remission rates albeit for a short duration, with ALL regimens having a higher remission percentage among both adults and children. Here we present a case of BPDCN without skin involvement.