Dendritic Cell Leukemia: a Review (original) (raw)
Related papers
Blastic Plasmacytoid Dendritic Cell Neoplasm: Update on Molecular Biology, Diagnosis, and Therapy
Cancer Control Journal of the Moffitt Cancer Center, 2014
Background: 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. Methods: 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. Results: 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. Conclusions: 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.
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.
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.
Blastic Plasmacytoid Dendritic Cell Neoplasm: A Rare Case Report with Literature Review
Indian Journal of Medical and Paediatric Oncology
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic neoplasm for which there are no effective therapies. We present a 70-year-old male patient with multiple reddish painless, nonpruritic, and nonpedunculated nodules over the trunk, forearm, and thighs for a duration of 3 months. The nodules measured 0.5 to 2 cm in diameter. The peripheral smear findings were within normal limits. Excision biopsy was performed. Histomorphology and immunohistochemistry (CD123, CD 56, CD4, HLA-DR, CD43, and CD68) confirmed the diagnosis of BPDCN. Findings of marrow aspiration, biopsy and imaging studies were within normal limits. Patient demonstrated a good response with complete disappearance of all nodules by initial 2 weeks of therapy with a modified Berlin–Frankfurt–Munster (BFM) acute lymphoblastic leukemia (ALL) protocol and has completed 8 doses (LSAP [lincosamides, streptogramins A and pleuromutilins chemotherapy], 5,000 units/m2). The patient tolerated protocol extreme...
Clinicopathologic retrospective analysis of blastic plasmacytoid dendritic cell neoplasms
Postepy dermatologii i alergologii, 2018
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is one of the aggressive rare hematopoietic malignancies with predilection to the skin, primarily found in adults. The precise incidence of BPDCN is difficult to estimate due to constantly changing nomenclature and lack of precise defining criteria prior to the 2008 WHO classification system. There are not many cases described in the literature, what makes the diagnostic process challenging. Skin lesions such as erythematous infiltrates and nodules are usually the first manifestation of the disease. Therefore, in doubtful diagnostic cases, dermatologists should perform histopathological and immunohistochemistry examinations along with hematological and oncological cooperation, as early diagnosis and appropriate treatment is essential for improvement of the disease course. This analysis, despite the small number of patients may provide useful information on the clinical and histopathological features of this rare malignancy.
Annals of dermatology, 2015
Blastic plasmacytoid dendritic cell neoplasm (BPDCN), which is derived from the precursor of plasmacytoid dendritic cells, is a rare and highly aggressive hematologic malignancy. It has only recently been recognized as a distinct entity. BPDCN characteristically has a predilection for cutaneous involvement. The aim of this study was to describe the clinical and pathological features of BPDCN, and to review the treatment courses to analyze the prognosis and the optimal therapeutic approach. We retrospectively reviewed seven BPDCN cases registered in the Samsung Medical Center database between January 2010 and December 2014. The median age of the patients was 52 years (range, 18~79 years), and six patients were male. The clinical staging was as follows: skin (n=5), lymph node (n=6), bone marrow (n=4), and peripheral blood (n=2). The skin manifestations were bruise-like tumefaction (n=4), erythematous nodule (n=4), or multiple erythematous papules (n=1). The pathological evaluation rev...
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...
Oncotarget, 2015
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of leukemia/lymphoma, whose diagnosis can be difficult to achieve due to its clinical and biological heterogeneity, as well as its overlapping features with other hematologic malignancies. In this study we investigated whether the association between the maturational stage of tumor cells and the clinico-biological and prognostic features of the disease, based on the analysis of 46 BPDCN cases classified into three maturation-associated subgroups on immunophenotypic grounds. Our results show that blasts from cases with an immature plasmacytoid dendritic cell (pDC) phenotype exhibit an uncommon CD56- phenotype, coexisting with CD34+ non-pDC tumor cells, typically in the absence of extramedullary (e.g. skin) disease at presentation. Conversely, patients with a more mature blast cell phenotype more frequently displayed skin/extramedullary involvement and spread into secondary lymphoid tissues. Despite the dismal outc...
Blastic Plasmacytoid Dendritic Cell Neoplasm in a Pediatric case -A Rare Case Report
IOSR Journals , 2019
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare CD4+ CD56+ myeloid malignancy that is challenging to diagnose and treat. BPDCN predominantly affects skin followed by blood/ bone marrow and later progresses to leukemia. It presents as nonspecific cutaneous lesions with or without extra-cutaneous manifestations, thereby forming a close differential diagnosis for hematodermic malignancies. We present a case of a 2.5-year-old child with blasticplasmacytoid dendritic cell neoplasm which is an extremely rare hematological malignancy with a distinct treatment protocol.