Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is an extremely rare invasive tumor with poor prognosis.The common clinical manifestations of BPDCN include skin injury,bone marrow involvement,and tumor cell spread.BPDCN is often misdiagnosed as other diseases and its diagnosis often requires a combination of clinical manifestations,imaging,histology,and immunophenotyping.Among them,immunophenotyping is crucial for the diagnosis of BPDCN.Although BPDCN is rare and no consensus has been reached on first-line treatment option,new drugs and options for treating this disease have emerged with the development of new drugs and increased awareness of BPDCN.This article reviews the research background,the origin of blastic plasmacytoid dendritic cells,and the recent research progress in the pathogenesis,diagnosis and differential diagnosis,treatment,and prognosis of BPDCN.
Humans ; Dendritic Cells ; Prognosis ; Diagnosis, Differential ; Immunophenotyping ; Skin Neoplasms/diagnosis* ; Hematologic Neoplasms/pathology*

No abstract available.
Adult ; Antigens, CD4/*metabolism ; Antigens, CD56/*metabolism ; Bone Marrow/metabolism/pathology ; Dendritic Cells/cytology/*metabolism ; Diagnostic Errors ; Exons ; Female ; Flow Cytometry ; Gene Rearrangement ; Hematologic Neoplasms/diagnosis ; Histone-Lysine N-Methyltransferase/genetics ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; Leukemia, Myeloid, Acute/*diagnosis ; Male ; Middle Aged ; Myeloid-Lymphoid Leukemia Protein/genetics ; Real-Time Polymerase Chain Reaction ; Sequence Analysis, DNA ; Transcription Factors/genetics ; Translocation, Genetic

Hematologic Neoplasms ; diagnosis ; pathology ; Humans

Dendritic Cells ; pathology ; Hematologic Neoplasms ; diagnosis ; therapy ; Humans

No abstract available.
Acute Disease ; Adult ; Antigens, CD4/metabolism ; Antigens, CD45/metabolism ; Antigens, CD56/metabolism ; Bone Marrow Cells/cytology ; Flow Cytometry ; Hematologic Neoplasms/*diagnosis/pathology ; Humans ; Interleukin-3 Receptor alpha Subunit/metabolism ; Lymph Nodes/metabolism/pathology ; Male ; Tomography, X-Ray Computed

BACKGROUND: Therapy-related myeloid neoplasms (t-MN) occur as late complications of cytotoxic therapy. This study reviewed clinical and cytogenetic characteristics of patients with t-MN at a single institution in Korea. METHODS: The study subjects included 39 consecutive patients diagnosed with t-MN. Each subject's clinical history of previous diseases, treatments, and laboratory data was reviewed, including cytogenetics. The primary diagnosis was hematologic malignancy in 14 patients and solid tumor in 25 patients. RESULTS: Therapy-related acute myeloid leukemia (t-AML, 66.7%) was found to be more common than therapy-related myelodysplastic syndrome (t-MDS). Primary hematologic malignancies that were commonly implicated included mature B-cell neoplasm and acute leukemia. Breast cancer was the most common primary solid tumor. The mean time interval from cytotoxic therapy initiation to t-MN detection was 49 months. Chromosomal aberrations were observed in 35 patients, and loss of chromosome 5, 7, or both accounted for 41% of all cases. Balanced rearrangements occurred in 13 patients; these patients showed shorter latency intervals (mean, 38 months) than patients with loss of chromosome 5 or 7 (mean, 61 months). CONCLUSIONS: In this study, we determined the clinical and cytogenetic characteristics of Korean patients with t-MN. Although our results were generally consistent with those of previous reports, we found that t-MN resulting from de novo leukemia was common and that t-AML was more common than t-MDS at presentation. Multi-institutional studies involving a larger number of patients and additional parameters are required to investigate the epidemiology, genetic predisposition, and survival rate of t-MN in Korea.
Adolescent ; Adult ; Aged ; Antineoplastic Agents/*adverse effects/therapeutic use ; Asian Continental Ancestry Group ; Bone Marrow/pathology ; Breast Neoplasms/drug therapy/pathology/radiotherapy ; Child ; Child, Preschool ; Chromosome Aberrations ; Chromosomes, Human, Pair 5 ; Chromosomes, Human, Pair 7 ; Female ; Hematologic Neoplasms/drug therapy/pathology/radiotherapy ; Humans ; Karyotyping ; Leukemia, Myeloid, Acute/*diagnosis/etiology/genetics ; Male ; Middle Aged ; Myelodysplastic Syndromes/*diagnosis/etiology/genetics ; Neoplasms, Second Primary/*diagnosis/etiology/genetics ; Republic of Korea ; Young Adult

Adolescent ; Adult ; Bone Marrow ; pathology ; Bone Marrow Examination ; methods ; Child ; Child, Preschool ; Female ; Hematologic Neoplasms ; diagnosis ; pathology ; Humans ; In Situ Hybridization, Fluorescence ; Male ; Middle Aged ; Retrospective Studies

CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Hematologic Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Leukemia, Myeloid ; pathology ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Skin Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery

OBJECTIVETo study the clinicopathologic features and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm.

METHODSThe clinical, morphology and immunophenotypic features were analyzed in 3 cases of blastic plasmacytoid dendritic cell neoplasm, with review of literature.

RESULTSThe pathologic changes of these tumors accorded with that of blastic plasmacytoid dendritic cell neoplasm, and they also had new characteristics, including lineage other than T, B, myeloid and NK cells, and immunophenotypes of CD56(+) CD4(-) CD123(+) TdT(+) CD43(+) CD68(+) , CD56(+) CD4(+) CD123(-) TdT(+) CD43(+) CD68(-) and CD56(+) CD4(+) CD123(-/+) TdT(-) CD43(+) CD68(+) in the 3 cases, respectively. Bone marrow involvement was found 5 years later in case 1, and was then stable after chemotherapy; case 2 and case 3 were died 5 and 2 months after diagnosis, respectively.

CONCLUSIONBlastic plasmacytoid dendritic cell neoplasm is a heterogeneous group of lymphoproliferative disorders, with different clinical, morphologic and immunophenotypic features.

Adolescent ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bleomycin ; therapeutic use ; CD56 Antigen ; metabolism ; Cyclophosphamide ; therapeutic use ; Dendritic Cells ; metabolism ; pathology ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Hematologic Neoplasms ; drug therapy ; metabolism ; pathology ; Humans ; Interleukin-3 Receptor alpha Subunit ; metabolism ; Leukemia, Myeloid ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; Male ; Middle Aged ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Prednisone ; therapeutic use ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; Treatment Outcome ; Vincristine ; therapeutic use

BACKGROUND: The incidence of bone marrow (BM) metastasis might be related with the occurrence of malignant tumors in ethnic groups. So, we investigated the type and the frequency of metastatic tumors of BM and analyzed the clinicopathologic variables of BM metastasis. METHODS: This study included 932 cases of primary malignant tumor which were requested for BM study from January 1995 to June 2006 in Chonnam National University Hospital and Chonnam National University Hwasun Hospital. Peripheral blood smears (PBS); aspirates, touch prints, and trephine biopsies of BM; and medical records including other laboratory test results were reviewed. RESULTS: Overall frequency of BM metastasis was 11.9% (111/932). Primary tumors with BM involvement in children comprised neuroblastoma (74.1%), rhabdomyosarcoma (7.4%), and malignant lymphoma (7.4%). For adult patients, they consisted of malignant lymphoma (56.0%), gastrointestinal cancer (20.2%), and lung cancer (6.0%). In the case of malignant lymphoma, diffuse large cell lymphoma was the most frequent one. Laboratory findings of patients with BM metastasis commonly showed anemia and thrombocytopenia; in addition, serum LD, ALP, AST and ALT were elevated in 81.5% (75/92), 63.4% (59/93), 63.5% (61/96) and 33.3% (32/96), respectively. Leukoerythroblastosis was observed only in 19.8% (22/111) on PBS examination. CONCLUSIONS: The most common non-hematopoietic metastatic tumor was neuroblastoma in children and gastrointestinal tumors in adults. Leukoerythroblastosis, anemia, and the elevation of serum LD, ALP, and AST were useful markers for the prediction of BM metastasis.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Bone Marrow Examination ; Bone Marrow Neoplasms/diagnosis/pathology/*secondary ; Child ; Child, Preschool ; Female ; Gastrointestinal Neoplasms/pathology ; Hematologic Tests ; Humans ; Infant ; Male ; Middle Aged ; Neuroblastoma/pathology