Natural killer cells 56bnght16- have higher counts in the umbilical cord blood than in the adult peripheral blood

ABSTRACT Introduction and hypothesis: Umbilical cord blood (UCB) is an alternative source of hematopoietic stem cells for allogeneic hematopoietic stem cell transplantation in the absence of a compatible donor. The UCB transplantation has a lower incidence of chronic graft versus host disease (GvHD), but is associated with slower engraftment and slower immune reconstitution, compared to other sources. Dendritic cells (DCs) and Natural Killer cells (NKs) play a central role in the development of GvHD and the graft versus leukemia (GvL) effect, as well as in the control of infectious complications. Method: We quantified by multiparametric flow cytometry monocytes, lymphocytes, NK cells, and DCs, including their subsets, in UCB samples from 54 healthy newborns and peripheral blood (PB) from 25 healthy adult volunteers. Results: In the UCB samples, there were higher counts of NK cells 56bright16- (median 0.024 × 109/L), compared to the PB samples (0.012 × 109/L, p < 0.0001), NK 56dim16bright (median 0.446 × 109/L vs. 0.259 × 109/L for PB samples, p = 0.001) and plasmacytoid dendritic cells (pDCs, median 0.008 × 109/L for UCB samples vs. 0.006 × 109/L for PB samples, p = 0.03). Moreover, non-classic monocyte counts were lower in UCB than in PB (median 0.024 × 109/L vs. 0.051 × 109/L, respectively, p < 0.0001). Conclusion: In conclusion, there were higher counts of NK cells and pDCs and lower counts of non-classic monocytes in UCB than in PB from healthy individuals. These findings might explain the lower incidence and severity of chronic GvHD, although maintaining the GvL effect, in UCB transplant recipients, compared to other stem cell sources.

Clinical Characteristics and Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm / 中国实验血液学杂志

OBJECTIVE@#To explore the clinical manifestations, diagnosis, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN).@*METHODS@#The clinical features, bone marrow morphology and immunophenotyping, treatment and prognosis of 4 patients with BPDCN were analyzed retrospectively.@*RESULTS@#4 patients had bone marrow， spleen and lymph nodes involvement， 2 patients had skin lesions， and 3 patients had central nervous system infiltration． Tailing phenomenon of abnormally cells could be seen in bone marrow. The immunophenotyping showed that CD56, CD4 and CD123 expression was observed in 4 patients， and CD304 in 3 patients． One patient refused chemotherapy and died early. Both patients achieved complete remission after the initial treatment with DA+VP regimen, 1 of them achieved complete remission after recurrence by using the same regimen again． One patient failed to respond to reduced dose of DA+VP chemotherapy, and then achieved complete remission with venetoclax+azacitidine.@*CONCLUSION@#The malignant cells in BPDCN patients often infiltrate bone marrow, spleen and lymph nodes, and have specical phenotypes, with poor prognosis. The treatment should take into account both myeloid and lymphatic systems. The treatment containing new drugs such as BCL-2 inhibitors combined with demethylation drugs is worth trying.

Clinical Analysis of Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm / 中国实验血液学杂志

OBJECTIVE@#To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm(BPDCN).@*METHODS@#Clinical data of 5 patients diagnosed with BPDCN in Wuhan First Hospital and Wuhan Tongji Hospital from June 2016 to November 2021 were retrospectively analyzed.@*RESULTS@#Among the 5 patients, 3 were male and 2 were female, with a median age of 28(10-52) years old. Four patients showed obvious skin damage at the initial diagnosis; the other one showed clinical manifestations of acute leukemia rather than obvious skin damage at the initial diagnosis, but infiltrated skin when the disease relapsed after treatment. Other infiltration sites of lesions included bone marrow (2/5), peripheral blood (2/5), lymph nodes (3/5), liver and spleen (2/5). All patients had no clinical manifestation of central nervous system infiltration. Tumor cell specific immune markers CD4, CD56, CD123 were all positive, and the median Ki-67 index was 70%. TET2, ASXL1 and NRAS gene mutations were found respectively in 3 patients by next-generation sequencing technique (NGS). ALL-like, AML-like and invasive NK/T cell lymphoma-like first-line induction chemotherapy regimens were used for the patients. One patient died of severe complications during the early stage of chemotherapy, 3 patients were evaluated as CR, and 1 patient was evaluated as PR. 2 patients were recurred and progressed after induction of chemotherapy, and one of them was evaluated as CR after re-treatment. One patient received autologous hematopoietic stem cell transplantation (auto-HSCT) and got long-term survival (OS 87 months). 3 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT), of which one died of transplantation related complications, and 2 cases survived. The median follow-up time of 4 patients with evaluable efficacy was 28.5(9-84) months, the median OS time was 31.5(10-87) months.@*CONCLUSION@#BPDCN is a highly heterogeneous malignant tumor with a poor prognosis. HSCT, especially allo-HSCT can significantly improve the prognosis of BPDCN patients.

Blastic plasmacytoid dendritic cell neoplasm: A clinico-pathological retrospective analysis of thirteen cases / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinicopathological features of blastic plasmacytoid dendritic cell neoplasm (BPDCN).@*METHODS@#A total of 13 cases of BPDCN diagnosed in Peking University First Hospital from January 2013 to March 2022 were collected. The clinical features, histopathological characteristics, immunophenotypes and prognosis of the patients were analyzed retrospectively, and the related literatures was reviewed as well.@*RESULTS@#Among the 13 patients, 11 were male and 2 were female, with a median age of 62 years (ranging from 5 to 78 years). Among them, single organ involvement occurred in 5 cases, all of which presented with skin lesions. Two or more organs were involved in other 8 cases (single organ with bone marrow involved in 3 cases; skin, bone marrow and lymph node involved simultaneously in 3 cases; skin, bone marrow, lymph node and spleen involved simultaneously in 2 cases). Histopathologically, it was characterized by the proliferation of medium to large atypical blastic cells, which infiltrated the whole thickness of dermis. When involved, the bone marrow lesions mainly appeared in a diffuse pattern, while the lymph node structure was usually destroyed, and the red pulp of the affected spleen was diffusely invaded. Immunohistochemical staining showed that all the 13 cases were positive for CD4, CD56, and CD123 (13/13) in varying degrees. All the 9 cases expressed TCL1 (9/9). Variable expression of CD68 (KP1) (8/13), TdT (7/12), CD117 (2/6), and high Ki-67 proliferation index (40%~80%) were showed. The neoplastic cells lacked expressions of CD20, CD3, MPO, CD34, or CD30; EBER in situ hybridization were negative (0/9). After definite diagnosis, 6 cases received chemotherapy, among which 1 received adjuvant radiotherapy, and 2 received subsequent bone marrow transplantation. Another 2 cases only received maintenance treatment. The median follow-up time was 14 months (ranging from 6 to 36 months), 5 patients died of the disease (6 to 18 months), 3 patients survived (7 to 36 months up to now), and the remaining 5 patients lost follow-up.@*CONCLUSION@#BPDCN is a rare type of malignant lymphohematopoietic tumor with aggressive behavior and poor prognosis. The diagnosis should be made combining clinical features, histopathology, and immunohistochemical phenotype. Attention should be paid to differentiating BPDCN from other neoplasms with blastoid morphology or CD4+CD56+ tumors.

Chemotherapy Combined with Venetoclax Followed by Allo-Hematopoietic Stem Cell Transplantation for Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm / 中国实验血液学杂志

OBJECTIVE@#To investigate the efficacy and safety of chemotherapy combined with venetoclax followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT) for the treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN).@*METHODS@#The clinical data of 3 patients with BPDCN undergoing allo-HSCT in Department of Hematology, Wuhan First Hospital from July 2017 to November 2021 were collected and retrospectively analyzed.@*RESULTS@#Among the 3 patients, there were 1 male and 2 females, aged 27-52 years old. Skin lesions were observed during initial diagnosis, and it could also be characterized by acute leukemia. Characteristic molecular markers of tumor cells, such as CD4, CD56, CD123, and CD303 were positive. In addition, the expression detection of Bcl-2 in 3 patients were positive. Chemotherapy combined with venetoclax in the initial induction of chemotherapy (1 case) or disease recurrence and progress (2 cases) was performed. There were 2 cases evaluated as complete remission (CR) and 1 case as partial remission (PR) before allo-HSCT. The patients all received a nonmyeloablative conditioning without total body irradiation (TBI). The prevention programme of graft-versus-host disease (GVHD) was antithymocyte globulin + mycophenolate mofetil + cyclosporin A/FK506 ± methotrexate. The number of mononuclear cell (MNC) count was (16.73-18.35)×108/kg, and CD34+ cell count was (3.57-4.65)×106/kg. The 3 patients were evaluated as CR after allo-HSCT (+21 to +28 d), the donor-recipient chimerism rate was 100%, and Ⅲ-Ⅳ GVHD was not observed. One patient died at +50 d after transplantation, two patients were followed up for 28 months and 15 months, respectively, and achieved disease-free survival (DFS).@*CONCLUSIONS@#BPDCN is a highly aggressive malignant tumor with poor prognosis. Chemotherapy combined with venetoclax followed by allo-HSCT may lead to long-term DFS or even cure. Post-transplant maintenance is still unclear.

Effect of Plasmacytoid Dendritic Cell Dose in Grafts on CMV Infection after Allogeneic Hematopoietic Stem Cell Transplantation / 中国实验血液学杂志

OBJECTIVE@#To investigate the correlation between plasmacytoid dendritic cell (pDC) dose in grafts and the occurrence of cytomegalovirus (CMV) infection after allogeneic hematopoietic stem cell transplantation (allo-HSCT).@*METHODS@#The clinical data of 80 children who received allo-HSCT in Children's Hospital of Soochow University from August 20, 2020 to June 11, 2021 were retrospectively analyzed. Proportions of DC subsets and T-cell subsets in grafts were detected by flow cytometry in order to calculate infused cell dose of each cell. Weekly monitoring of CMV-DNA copies in peripheral blood for each child were performed after transplantation. The last follow-up date was December 31, 2021.@*RESULTS@#All the children gained hematopoietic reconstitution. CMV infection was observed in 51 children (63.8%±5.4%) within the first 100 days after transplantation, including 2 cases developing CMV disease. Univariate analysis indicated that infused doses of DC and pDC were significantly associated with CMV infection within 100 days after allo-HSCT (P <0.05). Multivariate analysis indicated that a high dose infusion of pDC was an independent protective factor for CMV infection within 100 days after allo-HSCT (P <0.05). By the end of follow-up, 7 children died of transplantation-related complications, including 2 deaths from CMV disease, 2 deaths from extensive chronic graft-versus-host disease, and 3 deaths from capillary leak syndrome. The overall survival rate was 91.2%.@*CONCLUSION@#The pDC in grafts may be associated with early infection of CMV after allo-HSCT, while a high infused pDC dose may serve as a protective factor for CMV infection after transplantation.

Application study on adoptive transfusion of tolerogenic dendritic cells in promoting immune tolerance of liver transplantation in rat models / 器官移植

Objective To investigate the role of tolerogenic dendritic cell (tolDC) in inducing immune tolerance in liver transplantation. Methods Liver transplantation rat models of spontaneous tolerance [Brown Norway (BN)→Lewis, tolerance group, n=6] and acute rejection (AR) (Lewis→BN) were established. In AR rat models, tolDC transfusion was performed in the study group (tolDC group, n=6) and no intervention was given in the control group (AR group, n=6). The survival time of rats in each group was observed. The transplant liver tissues of rats were prepared for pathological examination in each group. The expression of myeloid dendritic cell (mDC) and plasmacytoid dendritic cell (pDC) in rat peripheral blood, transplant liver, spleen and lymph nodes in each group was detected by flow cytometry. The expression levels of serum interleukin (IL)-10 and interferon (IFN)-γ in each group were measured by enzyme-linked immune absorbent assay. Results Pathological manifestations of rats in the AR group mainly included inflammatory cell infiltration and tissue structural disorder in transplant liver, and the survival time was 7-14 d. In the tolDC and tolerance groups, the transplant liver tissues were almost normal, and the longest survival time exceeded 100 d. Compared with the AR group, the expression levels of CD11+mDC in peripheral blood, transplant liver, spleen and lymph nodes of rats were significantly down-regulated in the tolerance and tolDC groups (all P < 0.05), and those of CD86 and major histocompatibility complex (MHC)Ⅱon the surface of CD11+mDC were also significantly down-regulated (all P < 0.05). Compared with the AR group, the expression levels of pDC in peripheral blood, transplant liver, spleen and lymph nodes of rats were significantly up-regulated in the tolerance and tolDC groups (all P < 0.05), whereas those of MHCⅡon the surface of pDC were all significantly down-regulated (all P < 0.05). Compared with the AR group, the expression levels of serum IL-10 were significantly up-regulated, and IFN-γ were significantly down-regulated in the tolerance and tolDC groups (all P < 0.05). Conclusions As tolDC subsets, mDC and pDC play a positive role in regulating the incidence of graft immune tolerance in rats after liver transplantation.

Arsenic trioxide induces regulatory functions of plasmacytoid dendritic cells through interferon- inhibition

Arsenic trioxide (AsO) is recently found to have therapeutic potential in systemic sclerosis (SSc), a life-threatening multi-system fibrosing autoimmune disease with type I interferon (IFN-I) signature. Chronically activated plasmacytoid dendritic cells (pDCs) are responsible for IFN-I secretion and are closely related with fibrosis establishment in SSc. In this study, we showed that high concentrations of AsO induced apoptosis of pDCs mitochondrial pathway with increased BAX/BCL-2 ratio, while independent of reactive oxygen species generation. Notably, at clinical relevant concentrations, AsO preferentially inhibited IFN- secretion as compared to other cytokines such as TNF-, probably due to potent down-regulation of the total protein and mRNA expression, as well as phosphorylation of the interferon regulatory factor 7 (IRF7). In addition, AsO induced a suppressive phenotype, and in combination with cytokine inhibition, it down-regulated pDCs' capacity to induce CD4 T cell proliferation, Th1/Th22 polarization, and B cell differentiation towards plasmablasts. Moreover, chronically activated pDCs from SSc patients were not resistant to the selective IFN- inhibition, and regulatory phenotype induced by AsO. Collectively, our data suggest that AsO could target pDCs and exert its treatment efficacy in SSc, and more autoimmune disorders with IFN-I signature.

Neoplasia blástica de células dendríticas plasmocitoides variante leucemia aguda: reporte de un caso / Blastic plasmacytoid dendritic cell neoplasm variant acute leukemia: case report

RESUMEN La neoplasia blástica de células dendríticas plasmocitoides (NBCDP) es una malignidad hematológica poco frecuente y generalmente agresiva, por lo cual se requiere su reconocimiento precoz. A continuación, se describe el curso clínico prolongado de un paciente masculino de 60 años con NBCDP procedente de Venezuela, en cuyos hallazgos más relevantes destacó la presencia de lesiones cutáneas, organomegalias, infiltración de la médula ósea y del sistema nervioso central. Posterior al diagnóstico se indicó quimioterapia sistémica, no obstante, el paciente falleció por complicaciones respiratorias durante la fase de inducción del tratamiento. En esta enfermedad es necesario establecer el diagnóstico diferencial con trastornos linfoproliferativos, leucemias linfoides y mieloides agudas, constituyendo el análisis morfológico de las células neoplásicas un aspecto importante para una adecuada orientación diagnóstica.

ABSTRACT Blastic plasmacytoid dendritic cell blast neoplasm (BPDCN) is a rare and generally aggressive hematologic malignancy, requiring early recognition. Below is a description of the prolonged clinical course of a 60-year-old male patient with BPDCN from Venezuela, whose most relevant findings highlighted the presence of skin lesions, organomegaly, infiltration of the bone marrow and central nervous system. Systemic chemotherapy was prescribed after diagnosis; however, the patient died of respiratory complications during the induction phase of treatment. In this disease, it is necessary to establish the differential diagnosis with lymphoproliferative disorders, acute lymphoid and myeloid leukemias. The morphological analysis of neoplastic cells is, thus, an important aspect toward proper diagnostic guidance.

New progression in the therapy of blastic plasmacytoid dendritic cell neoplasms / 国际肿瘤学杂志

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a kind of rare and highly invasive hematological malignancy. Because of its low incidence, there is still no consensus on its standard treatment. For young patients, high intensity chemotherapy combined with hematopoietic stem cell transplantation is often used. Elderly patients who cannot accept hematopoietic stem cell transplantation receive low intensity chemotherapy. In December 2018, tagraxofusp, a new targeted drug, was approved by the U. S. Food and Drug Administration specially for treatment-naive and previously-treated BPDCN patients (age≥2 years old). Some targeted drugs, such as venetoclax and daratumumab, have certain effects on the treatment of BPDCN. The efficacies of PD-1/PDL-1 inhibitors and anti CD123 chimeric antigen receptor T cell immunotherapy need further researches.

Progress of blastic plasmacytoid dendritic cell neoplasms / 白血病·淋巴瘤

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a kind of highly malignant tumor in the hematological system, which is characterized with low incidence, diverse clinical manifestations, strong invasiveness and poor prognosis, however, currently there has no standard treatment yet. This paper reviews the recent research progress of BPDCN.

Bumps of blood cells: Blastic Plasmacytoid Dendritic Cell Neoplasm in an elderly Filipino

Introduction@#Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy derived from the precursors of plasmacytoid dendritic cells. This malignancy presents with various noticeable cutaneous lesions and usually occurs in elderly males. Cutaneous manifestations usually precede leukemic dissemination to the lymph nodes, bone marrow, and peripheral blood which is associated with poor prognosis.@*Case presentation@#We present a case of a 60-year-old Filipino male with a four-month history of multiple hyperpigmented, reddish brown, firm, fixed, non-tender cutaneous nodules on the extremities, trunk, chest, and face. Two large masses was also noted on the left arm and left upper back..Tissue biopsy of the cutaneous mass showed Immunohistochemical stain findings positive for LCA, CD68, CD4, CD56, and CD123 which are compatible with BPDCN. Patient was initially asymptomatic with relatively normal blood count and was treated supportively but serial blood count monitoring showed worsening with progression to acute myelogenous leukemia. Patient was then started on the 7+3 protocol of cytarabine and idarubicine which provided flattening of the cutaneous nodules and improvement of blood counts. However, due to complications of the disease and the treatment, the patient succumbed to severe pulmonary infection and sepsis.@*Discussion@#Due to the varied, non-specific cutaneous manifestations and the similarity in the morphology of the skin lesions with other cutaneous conditions along with the rarity of this disease, there is difficulty in establishing the diagnosis of BPDCN as well as standardizing its treatment. Immunohistochemical stains play an important role in confirming the diagnosis as well as ruling out other differential diagnoses to tailor appropriate treatment.@*Conclusion@#Blastic plasmacytoid dendritic cell neoplasm (BPDCN) generally has a poor prognosis owing to the rapidity of its spread to the bone marrow and peripheral blood. Early diagnosis is essential to initiate early therapy and prevent progression.

New progression in the therapy of blastic plasmacytoid dendritic cell neoplasms / 国际肿瘤学杂志

Blastic plasmacytoid dendritic cell neoplasm (BPDCN)is a kind of rare and highly invasive hematological malignancy. Because of its low incidence,there is still no consensus on its standard treatment. For young patients,high intensity chemotherapy combined with hematopoietic stem cell transplantation is often used. Elderly patients who cannot accept hematopoietic stem cell transplantation receive low intensity chemo-therapy. In December 2018,tagraxofusp,a new targeted drug,was approved by the U. S. Food and Drug Administration specially for treatment-naive and previously-treated BPDCN patients (age≥2 years old). Some targeted drugs,such as venetoclax and daratumumab,have certain effects on the treatment of BPDCN. The efficacies of PD-1 / PDL-1 inhibitors and anti CD123 chimeric antigen receptor T cell immunotherapy need further researches.

The Role of Plasmacytoid Dendritic Cells in Gut Health

Plasmacytoid dendritic cells (pDCs) are a unique subset of cells with different functional characteristics compared to classical dendritic cells. The pDCs are critical for the production of type I IFN in response to microbial and self-nucleic acids. They have an important role for host defense against viral pathogen infections. In addition, pDCs have been well studied as a critical player for breaking tolerance to self-nucleic acids that induce autoimmune disorders such as systemic lupus erythematosus. However, pDCs have an immunoregulatory role in inducing the immune tolerance by generating Tregs and various regulatory mechanisms in mucosal tissues. Here, we summarize the recent studies of pDCs that focused on the functional characteristics of gut pDCs, including interactions with other immune cells in the gut. Furthermore, the dynamic role of gut pDCs will be investigated with respect to disease status including gut infection, inflammatory bowel disease, and cancers.

Predictive value of peripheral blood myeloid dendritic cell in 7-day adverse cardiac events in patients with acute ST segment elevation myocardial infarction / 中国医师进修杂志

Objective To investigate the effect of peripheral blood MDC on predicting adverse cardiac events of patients with acute STEMI. Methods Thirty-nine patients with acute ST segment elevation myocardial infarction admitted in the People′s Hospital of Deyang from February 2016 to August 2017 were selected by random sampling. According to 7-day adverse cardiac events, the patients were divided into non-combination adverse cardiac events group (uncomMI group, 23 patients) and combination adverse cardiac events group (comMI group, 16 patients), and 24 healthy persons were selected as normal control group. The general situation, clinical characteristics and MDC, PDC, analysis of uncomMI group and comMI group in the 1st and 3rd day after the onset of acute ST segment elevation myocardial infarction was compared. Factors influencing adverse cardiac events in patients with acute ST segment elevation myocardial infarction were analyzed. Results The peripheral blood MDC and plasmacytoid dendritic cell (PDC) counts in patients with STEMI were significantly lower than those in normal controls in early time(at the time of day 1)(P<0.05), but the peripheral blood MDC and PDC counts in uncomMI group was not significantly different from that in normal control group at the time of day 3 (P>0.05). The peripheral blood MDC and PDC counts in comMI group were lower than those in uncomMI group and control group. The Logistic analysis showed that the elderly patients, total cholesterol (TC), MDC (at the time of day 3) were all risk factors of 7-day adverse cardiac events (P < 0.05). The receiver operating characteristic (ROC) results showed that the maximum area under curve(AUC) was 0.8286 when the MDC truncation point was 7 mg/L, and the sensitivity was 80.0% and the specificity was 85.7% . Conclusions For STEMI patients, MDC of peripheral blood is associated with 7-day adverse cardiac events. The percentage of MDC in peripheral blood of patients with acute STEMI has a certain clinical value in the diagnosis of 7-day adverse cardiac events. In addition, elderly patients with STEMI have an increased risk of 7-day adverse cardiac events.

Clinicopathological characteristics analysis on 10 cases of blastic plasmacytoid dendritic cell neoplasm / 临床与实验病理学杂志

Purpose To study the clinicopathological characteristics of blastic Plasmacytoid dendritic cell neoplasms, and improve the acknowledgement and diagnostic level. Methods The clinicopathological features, immunohistochemistry and EBV test results of 10 cases with BPDCN were analyzed retrospectively, with review of literature. Results Skin lesions were the first symptom in 10 patients, tumor cells expression of CD43, CD56, CD4 was positive in 8 cases, CD123 were positive in 5 cases, the T and B cell specific markers is not expression. EB virus encoded small RNA(EBER ) in situ hybridization is negative;no clonal rearrangement of B cell receptor(BCR) or T cell receptor(TCR) gene. Conclusion BPDCN is a rare malignant tumor of the skin, easy to involve the skin. It is necessary to combine clinical with pathological for definite diagnosis.

A case of blastic plasmacytoid dendritic cell neoplasm / 天津医药

@#Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic neoplasm with high malignancy and rapid progression. BPDCN is sensitive to chemotherapy, but it is prone to relapse and drug resistance, and the survival time is short. We report a case of BPDCN hospitalized in our department. In this case, the skin lesions were the first manifestation. The diagnosis of BPDCN was confirmed by the skin biopsy. Initially, this patient was sensitive to the lymphoma chemotherapy but the disease relapsed quickly, spreading to the lungs and mediastinum. Then, we treated the patient with myeloid regimen, which was no efficacy. The patient eventually died with an overall survival of 17 months.

Clinical characteristics of 6 patients with blastic plasmacytoid dendritic cell neoplasm / 中华血液学杂志

Objective@#To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm.@*Method@#Clinical records of 6 patients diagnosed with blastic plasmacytoid dendritic cell neoplasm in our hospital from January 2008 to May 2016 were collected and retrospectively analyzed.@*Results@#Six patients manifested with initial symptoms of skin lesions, other common symptoms included bone marrow involvement (5/6) , lymphadenectasis (4/6) , splenomegaly (4/6) , and hepatomegaly (3/6) . In addition, extra-nodal involvement except skin was also observed, including breast (1/6) , maxillary sinus (1/6) , vertebrae (1/6) , and central nervous system (1/6) . Characteristic immunophenotype, CD4, CD56, and CD123 were all positive. All these patients were treated with acute lymphoblastic leukemia type (ALL-type) chemotherapy and complete remission (CR) were reached in 4 patients. The median follow-up was 9.5 (7-37) months, median progression free survival was 7 months; while median overall survival was 9 months. A total of 3 patients died during the follow-up, which were all happened in the first year after diagnosis, and all resulted from the relapse or disease progression.@*Conclusion@#Blastic plasmacytoid dendritic cell neoplasm is highly aggressive, in which the skin lesions are always manifested as initial symptoms, and bone marrow involvement, lymphadenectasis, splenomegaly, and hepatomegaly is also common. Characteristic immunophenotype include the positivity of CD4, CD56, and CD123. Effective and standard therapy is limited in this disease, which indicates the poor prognosis.

Clinical features and treatment outcomes of blastic plasmacytoid dendritic cell neoplasm: a single-center experience in Korea

BACKGROUND/AIMS: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that typically presents in the form of skin manifestations with or without lymph node and bone marrow involvement. Given its rarity and recent recognition as a distinct pathological entity, no standard of treatment exists for this aggressive disease and its prognosis is particularly dismal. METHODS: We retrospectively analyzed clinical features and treatment outcomes of patients who were diagnosed with BPDCN between 2000 and 2014. RESULTS: Ten patients had a median age at diagnosis of 41 years (range, 18 to 79), and seven patients were male. Sites of disease involvement were the skin (n = 7), lymph node (n = 5), bone marrow (n = 2), liver (n = 2), spleen (n = 2), and soft tissue (n = 1). Intensified chemotherapy regimens such as hyperCVAD regimen (cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, cytarabine), and VPDL (vincristine, methylprednisolone, daunorubicin, L-asparaginase) were used as a first-line treatment. Although all patients treated with intensified chemotherapy showed an objective response (five patients with complete response) with median progression-free survival of 11.2 months (range 6.2 to 19.4), complete remission was not sustained for more than 2 years in any case. The response was relatively long-lived compared with previously reported CHOP (doxorubicin, cyclophosphamide, vincristine, prednisone)-like regimens, but the above regimens do not result in long-term remission. CONCLUSIONS: All patients treated with hyperCVAD or VPDL showed an objective response, but the duration of response was relatively short. Thus, the development of more effective induction as well as consolidation treatment strategy should be warranted to improve this rare disease entity.

Clinicopathological characteristics of blastic plasmacytoid dendritic cell neoplasm / 肿瘤研究与临床

Objective To improve the understanding of blastic plasmacytoid dendric cell neoplasm (BPDCN). Methods The clinicopathological features of 2 cases with BPDCN diagnosed in the Department of Pathology in Cancer Hospital of Chinese Academy of Medical Sciences and Peking Union Medical College were analyzed retrospectively. The relevant literatures were reviewed as well. Results The two BPDCN patients were both elderly men with skin nodules. The tumor cells infiltrated diffusely into the whole dermis with sparing the epidermis. BPDCN cells were small to medium size with round or oval irregular nuclei and small nucleoli. Numerous mitoses figures were seen. Immunohistochemistry stain showed that CD4 and CD56 were positive and CD20 and CD3 were negative in the 2 BPDCN cases. Only 1 case was positive for CD123. Conclusions BPDCN is a rare malignant hematopoietic tumor, which is likely to involve skin. The features of morphology and immunophenotype are crucial to make correct diagnosis.