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.

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.

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.

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.

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.

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.

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.

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.

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.

A Woman with Blastic Plasmacytoid Dendritic Cell Neoplasm / 대한피부과학회지

No abstract available.

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.

Follicular dendritic cell sarcoma: report of two cases and literature review / Sarcoma de células dendríticas foliculares: relato de dois casos e revisão da literatura

ABSTRACTFollicular dendritic cell sarcoma is a rare neoplasm, first described in 1986 by Monda. Case 1: A female patient, 50-year-old performed abdominal computed tomography scan that detected a tumor lesion of 8.0 cm in the mesentery. She underwent resection of the lesion. Microscopic examination revealed epithelioid neoplasm, interspersed with lymphocytes, and positive immunohistochemical staining for CD21 and CD35. The patient underwent adjuvant chemotherapy. Case 2: A male patient, 21-year-old presented right-sided neck mass measuring 7.0 cm. The biopsy revealed proliferation of spindle cells, interspersed with inflammatory infiltrate and storiform arrangement, and positive immunohistochemical staining for CD21 and CD23. The patient underwent neoadjuvant radiotherapy and surgical resection.

RESUMOSarcoma de células dendríticas foliculares é uma neoplasia rara, descrita pela primeira vez em 1986 por Monda. Caso 1: Paciente do sexo feminino, 50 anos, realizou tomografia computadorizada de abdômen que detectou lesão tumoral de 8,0 cm em mesentério. Foi submetida à ressecção da lesão. A microscopia revelou neoplasia epitelioide, com linfócitos de permeio e expressão imuno-histoquímica de CD21 e CD35. A paciente foi submetida à quimioterapia adjuvante. Caso 2: Paciente do sexo masculino, 21 anos, com massa cervical direita medindo 7,0 cm. A biópsia evidenciou proliferação de células fusiformes, com infiltrado inflamatório de permeio e arranjo estoriforme, com expressão imuno-histoquímica de CD21 e CD23. O paciente foi submetido a radioterapia neoadjuvante e ressecção cirúrgica.

Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive neoplasm classifi ed under “acute myeloid leukemia (AML) and related precursor neoplasm” by current WHO classifi cation. Elderly male are commonly affected with cutaneous lesion being the hallmark of disease presentation. The disease progresses rapidly and sooner or later involves bone marrow and peripheral blood. Cases presenting primarily as leukemia without cutaneous involvement is a rarity with about 29 cases reported in literature till date. Characteristic immunophenotype of CD4+/CD56+/− cells expressing antigens associated with plasmacytoid dendritic cells like CD123, TCL1, BDCA2/CD303, cutaneous lymphocyte-associated and interferon dependent molecule MxA, in absence of any other lineage specifi c marker confi rms the diagnosis. The disease has a poor survival and no standardized therapeutic strategy in the current scenario. A case of 25-year-male presenting with leukemic BPDCN without cutaneous involvement is presented here, who was treated with AML like protocol followed by hematopoietic stem cell transplantation, but succumbed to the disease within 8 months of diagnosis. The present case is being fi rst to be reported from India.

Blastic plasmacytoid dendritic cell neoplasm: Report of two pediatric cases.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of acute leukemia that typically follows a highly aggressive clinical course in adults, whereas experience in children with this disease is very limited. We report cases of two children in whom bone marrow showed infi ltration by large atypical monocytoid ‘blast-like’ cells which on immunophenotyping expressed CD4, CD56, HLA-DR and CD33 while were negative for CD34 other T-cell, B-cell and myeloid markers. The differential diagnoses considered were AML, T/NKcell leukemia and acute undifferentiated leukemia. Additional markers CD303/ BDCA-2 and CD123 which are recently validated plasmacytoid dendritic cell markers were done which helped us clinch the diagnosis of this rare neoplasm. An accurate diagnosis of BPDCN is essential in order to provide prompt treatment. Due to its rarity and only recent recognition as a distinct clinicopathological entity, no standardized therapeutic approach has been established for BPDCN.

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm in a Child / 임상소아혈액종양

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare hematological malignancy in children that is characterized by bruise-like skin lesions, with or without bone marrow involvement. Because the clinical course of BPDCN is highly aggressive and fatal in adults, allogeneic hematopoietic stem cell transplantation (HSCT) is recommended as the optimal treatment. Due to its rarity, the pediatric experience with BPDCN is limited and standard treatment has so far not been defined. We report a case of a 14-year-old boy with BPDCN involving the skin, bone marrow and lymph nodes. The patient was treated with high-risk group acute lymphoblastic leukemia protocol. He achieved a complete remission after induction chemotherapy and still maintains clinical remission without HSCT for 17 months after initial diagnosis.

Blastic plasmacytoid dendritic cell neoplasm: a case report and literature review / 白血病·淋巴瘤

Objective To provide new information for treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN).Methods Through one case report and literature review of 48 BPDCN cases were reviewed retrospectively.The clinical characteristics,treatment choices and prognosis were analyzed.Results BPDCN patients were mainly elderly males,mostly presented as skin rash and bone marrow infiltration.Immunophenotype was characteristically expressed as CD4,CD56 and CD123.Lymphoid-like regimens could induce higher response rate,lower relapse rate and longer overall survival compared with myeloid-like regimens.Allogeneic hematopoietic stem cell transplantation may provide long-term survival.At the onset of the disease,The counts of white blood cells (WBC) and blood platelet (Plt) may be correlated with inferior overall survival.Conclusions BPDCN is a disease with distinct clinical characteristics and immunophenotype.Lymphoid-like regimen may be the better treatment of choice,while allogeneic hematopoietic stem cell transplantation should be taken into account in the first complete remission for longterm survival.

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm in a Child / 임상소아혈액종양

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare hematological malignancy in children that is characterized by bruise-like skin lesions, with or without bone marrow involvement. Because the clinical course of BPDCN is highly aggressive and fatal in adults, allogeneic hematopoietic stem cell transplantation (HSCT) is recommended as the optimal treatment. Due to its rarity, the pediatric experience with BPDCN is limited and standard treatment has so far not been defined. We report a case of a 14-year-old boy with BPDCN involving the skin, bone marrow and lymph nodes. The patient was treated with high-risk group acute lymphoblastic leukemia protocol. He achieved a complete remission after induction chemotherapy and still maintains clinical remission without HSCT for 17 months after initial diagnosis.

Blastic Plasmacytoid Dendritic Cell Neoplasm: Analysis of Clinicopathological Feature and Treatment Outcome of Seven Cases

BACKGROUND: 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. OBJECTIVE: 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. METHODS: We retrospectively reviewed seven BPDCN cases registered in the Samsung Medical Center database between January 2010 and December 2014. RESULTS: 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 revealed dense diffuse or nodular infiltration of neoplastic cells, which were positive for CD4, CD56, and CD123 in the immunohistochemical analysis. Six patients received multiagent chemotherapy as the first-line treatment, alone (n=4), or followed by stem cell transplantation (SCT, n=1) or concurrent radiotherapy (n=1). The median progression-free survival after the first-line treatment was 6 months (range, 2~12 months). CONCLUSION: Three different skin manifestations were observed, with pathological features analogous to each other. All patients who received chemotherapy without SCT achieved partial or complete response but experienced relapse. Furthermore, they showed various clinical courses irrelevant to the cutaneous involvement.