scholarly journals The Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasms Presenting Leukemia Dissemination, Diagnosed By Nation-Wide Immunophenotyping for Pediatric Hematological Malignancy in Japan

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4966-4966
Author(s):  
Takao Deguchi ◽  
Nobutaka Kiyokawa ◽  
Yoshiko Hashii ◽  
Akiko M. Saito ◽  
Atsushi Manabe ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Myelogenous Leukemia ◽  
World Health ◽  
Diagnostic Strategy ◽  
Histopathologic Examination ◽  
Cutaneous Lesions ◽  
Pediatric Leukemia

Abstract [Background] According to the nation-wide retrospective study for pediatric leukemia phenotyping, immunological diagnostic committee of Japanese Pediatric Leukemia/Lymphoma Study Group (JPLSG) established diagnostic criteria and panel to pediatric hematological malignancies in 2008. Then Nation-wide standardized central immnophenotyping in Japan was started from 2011, and done in more than 2,500 cases by the end of 2014. Most cases were determined immunological diagnosis, but it was of note that we found 11 acute unclassified leukemia cases, including typical and atypical blastic plasmacytoid dendritic cell neoplasms (BPDCN). BPDCN is a rare subtype of leukemia/lymphoma, which has been categorized in the 2008 World Health Organization (WHO) classification of hematological diseases under acute myeloid leukemia and related precursor neoplasms. It was well known for high incidence of extra medullary regions. Most cases of BPDCN were found in elder people, so pediatric cases were extremely rare. We report here the characteristics of pediatric BPDCN cases, including clinical manifestations and peculiar immunophenotype. [Materials and Methods] Immunophenotyping was done on bone marrow (BM) aspiration samples according to diagnostic panel and criteria established by JPLSG immunological diagnostic committee. Immunophenotyping data from July 2011 to June 2015 were collected from JPLSG immunophenotyping centers and analyzed. BPDCN cases were screened by typically CD4+ 56+ HLA-DRhi profile. Other lineage markers such as CD7, CD34, CD13, CD33, CD36, CD64, CD123, and NG2 were also analyzed. As it is well known that existence of atypical phenotype in a substantial proportion, including absence of CD56 and/or CD4, immunophenotype data were prudently investigated and compared with 21 acute myelogenous leukemia (AML) presenting M5 characteristics in FAB criteria. [Result] Out of more than 2,500 cases, 4 cases were diagnosed as typical BPDCN (CD4+ 56+ HLA-DRhi), and 2 atypical BPDCN (1 CD56-, 1 CD4-) by immunophenotyping. All 6 cases presented leukemic dissemination, but only 4 typical cases indicated extra medullary regions. Gender ratio (M: F) was 3:1 and 0:2, and median age were 6.9 years (3.3 to 11.3) and 13.8 (12.8, 14.7) respectively. All 6 cases were negative for CD34 and CD13, and positive for NG2. CD123 was also positive in all (5/5) cases. CD33 and CD36 were positive in 5 cases respectively. It was of note that CD64, typical monocytic marker, was negative in all 6 cases although it was positive in all AML-M5 cases. All BPDCN cases could be distinguished with expression patterns of NG2, CD7, CD33, CD36 (positive) or CD13, CD64 (negative). Additionally, CD2 was positive in 3/6 cases, TdT was positive in 2/6 cases. CD117 was positive in 2 cases (1 typical, 1 atypical). [Discussion] BPDCN is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. Diagnosis of BPDCN has been usually made by histopathologic examination with cutaneous lesions, which is typically positive for interleukin-3 receptor (CD123), blood dendritic cell antigen 2 (BDCA2)/CD303, and TCL-1. Morphologic and immunophenotypic studies were usually performed on BM and/or peripheral blood prior to histopathologic examination, however diagnosis with these examinations as BPDCN is not so easy because of the lack of morphological peculiarity or traditional lineage-specific markers. Treatments for BPDCN patients have been also inconsistent, because there are no prospective clinical trial data to define the optimal frontline treatment. AML-like or acute lymphoblastic leukemia (ALL)-like regimens have been used for induction therapy, as well as lymphoma-like regimens. The difficulty of BPDCN diagnosis might affect to choose inconsistent frontline therapy. Recently some reports documented ALL oriented therapy indicated more effectiveness to BPDCN, so the diagnosis should be made precisely to evaluate the treatment effectiveness. Here we indicated new diagnostic strategy by immunophenotyping with NG2, CD7, CD33, CD36, CD13, and CD64 as well as CD4, CD56 and HLA-DR. This approach contributes not only to select treatment regimen but also to investigate molecular pathogenesis such as whole exome sequence. Disclosures No relevant conflicts of interest to declare.

scholarly journals A Case of Plasmacytoid Dendritic Cell Leukemia

2013 ◽  
Vol 59 (2) ◽  
pp. 111-114
Author(s):  
Judit Beáta Köpeczi ◽  
I Benedek ◽  
Erzsébet Benedek ◽  
Enikő Kakucs ◽  
Aliz Tunyogi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Initial Response ◽  
Skin Lesions ◽  
High Rate ◽  
Response To Treatment ◽  
Cell Leukemia ◽  
Cutaneous Lesions ◽  
Hla Dr

AbstractIntroduction: Plasmacytoid dendritic cell leukemia is a rare subtype of acute leukemia, which has recently been established as a distinct pathologic entity that typically follows a highly aggressive clinical course in adults. The aim of this report is to present a case of plasmacytoid dendritic cell leukemia due to its rarity and difficulty to recognize and diagnose it.Case report: We present a case of a 67 year-old man who presented multiple subcutaneous lesions on his face, neck, chest and upper extremities with reddish-brown, brown colour. In the bone marrow aspirate 83% of the blast cells were found. Immunophenotypically the blasts were positive for CD4, CD56, CD123 (high intensity), CD36, CD22, CD10 (10.42%), CD33, HLA-DR, CD7 (9.24%), CD38 (34.8%) and negative for CD13, CD64, CD14, CD16, CD15, CD11b, CD11c, CD3, CD5, CD2, CD8, CD19, CD20, CD34. The skin biopsy showed lymphohistiocytoid infiltration in the dermis. The patient was diagnosed with acute plasmacytoid dendritic cell leukemia and received polychemotherapy with rapid response of skin lesions and blastic infiltration of the bone marrow. After 3 courses of polychemotherapy the cutaneous lesions reappeared and multiplied. The blast infiltration in the bone marrow increased to 70%. A more aggressive polychemotherapy regimen was administered, but the patient presented serious complications (febrile neutropenia) and died in septic shock 8 months after the initiation of treatment.Conclusions: Immunophenotyping of blasts cells is indispensable in the diagnosis of plasmacytoid dendritic cell leukemia. The CD4+, CD56+, lin-, CD123 ++high, CD11c-, CD36+, HLA-DR+, CD34-, CD45+ low profile is highly suggestive for pDCL. The outcome of plasmacytoid dendritic cell leukemia is poor. Despite the high rate of initial response to treatment, early relapses occur and the patients die of disease progression.

scholarly journals Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases

2021 ◽  
Vol 9 (33) ◽  
pp. 10293-10299
Author(s):  
Jiang-Hong Guo ◽  
Hong-Wei Zhang ◽  
Li Wang ◽  
Wei Bai ◽  
Jin-Fen Wang
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Cell Neoplasm

Immunohistochemical analysis of CD123, CD56 and CD4 for the diagnosis of minimal bone marrow involvement by blastic plasmacytoid dendritic cell neoplasm

2013 ◽  
Vol 62 (5) ◽  
pp. 764-770 ◽  
Author(s):  
Keumrock Hwang ◽  
Chan-Jeoung Park ◽  
Seongsoo Jang ◽  
Hyun-Sook Chi ◽  
Joo-Ryung Huh ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Immunohistochemical Analysis ◽  
Cell Neoplasm

scholarly journals Developmental Ontogeny of Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Revealed By Recurrent High Burden Clonal Hematopoiesis, Including in "Skin-Only" Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2755-2755 ◽  
Author(s):  
Gabriel K Griffin ◽  
Katsuhiro Togami ◽  
Elizabeth A Morgan ◽  
Andrew A. Lane
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Malignant Transformation ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Tumor Burden ◽  
Skin Tumors ◽  
Skin Tumor ◽  
Clonal Hematopoiesis ◽  
Post Therapy

Abstract INTRODUCTION: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematologic neoplasm with poor outcomes and limited therapeutic options. BPDCN pathogenesis is not clear, given that half of patients present with skin tumors without overt bone marrow involvement ("skin-only" disease). Most patients with "skin-only" BPDCN will eventually develop disease progression with bone marrow infiltration. Accordingly, a model of BPDCN pathogenesis has been proposed wherein malignant transformation of a plasmacytoid dendritic cell first occurs in the skin and is followed by dissemination to the bone marrow. Formal evidence to support this model is lacking. METHODS: To study the developmental ontogeny of BPDCN, we performed DNA sequencing of diagnostic bone marrow and skin biopsies from 10 patients. Four patients had "skin-only" disease with negative marrow assessments by histology and flow cytometry (Fig 1A-B). Two patients had skin tumors with limited marrow involvement (≤10% of the cellularity) and 4 patients had skin tumors with extensive concurrent marrow involvement. Six of ten patients had marrow sequencing performed serially before and after therapy. Exon capture and amplicon-based targeted sequencing assays covering recurrently mutated genes in blood cancers were used. Whole exome sequencing (WES) was also performed on germline DNA, "uninvolved" marrow, and a skin tumor from one patient with "skin-only" BPDCN. RESULTS: All patients with "skin-only" disease and negative bone marrow assessment at diagnosis harbored high variant allele frequency (VAF) mutations in the bone marrow, consistent with clonal hematopoiesis (Fig 1C). Two patients with cutaneous disease and only limited marrow involvement also demonstrated multiple high VAF mutations in the marrow that exceeded the degree of BPDCN tumor involvement. Mutations observed in these patients' bone marrows included known pathogenic variants in genes recurrently mutated in BPDCN and other blood cancers, including ASXL1 (4/6 cases; 45-71% VAF), TET2 (4/6; 31-45% VAF), SF3B1 (1/6; 22% VAF), ZRSR2 (1/6; 86% VAF), CUX1 (1/6; 88% VAF), and EZH2 (1/6; 79% VAF). Post-therapy analysis also suggested underlying clonal hematopoiesis in BPDCN. In a patient with 10% bone marrow involvement by tumor at diagnosis, persistent high VAF TET2 and ZRSR2 mutations were noted post-therapy despite a reduction in tumor burden to <1% of the cellularity (Fig 1D, patient 5). Similarly, 2 patients with more extensive marrow involvement at diagnosis (>30% of the cellularity) showed persistence of pathogenic mutations in JAK2, SRSF2, and GNB1 post-therapy despite a reduction in the marrow tumor burden to <1% of the cellularity (Fig 1D, patients 4 and 8). Most marrow mutations were also detected in paired biopsies of skin tumors, suggesting clonal progression from a pre-malignant marrow precursor. To further validate the clonal relationship between these anatomic compartments in BPDCN, we performed WES in one patient with "skin-only" disease who showed identical ASXL1 and TET2 mutations in the marrow and skin at diagnosis. Interestingly, by WES, 87% of all somatic single nucleotide variants (SNVs) were unique to the skin tumor, 11% of SNVs were unique to the bone marrow, and only 2% were shared between both anatomic sites (Fig 1E). This supports a model of branching pre-malignant evolution in the marrow, with one sub-clone seeding the skin and acquiring additional mutations during malignant transformation. CONCLUSION: Clonal hematopoiesis is a defining feature of BPDCN. High VAF mutations in the bone marrow of BPDCN patients without overt tumor involvement likely reflects extensive pre-malignant clonality rather than infiltration by tumor cells arising in the periphery. In contrast to clonal hematopoiesis of indeterminant potential (CHIP), which usually has a single gene mutated at low VAF, uninvolved marrow in BPDCN often harbors multiple mutations at high clonal burden. These data suggest that the earliest events in BPDCN pathogenesis occur in hematopoietic progenitor cells, which then seed peripheral sites during complete malignant transformation. These findings also raise concern about the use of morphologically "normal" stem cells for autologous transplantation in patients with BPDCN. Disclosures Griffin: Moderna Therapeutics: Consultancy. Lane:N-of-one: Consultancy; Stemline Therapeutics: Research Funding.

Blastic Plasmacytoid Dendritic Cell Neoplasm: Report of Three Cases.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4703-4703
Author(s):  
Paola Carluccio ◽  
Mario Delia ◽  
Anna Mestice ◽  
Domenico Pastore ◽  
Alessandra Ricco ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cells ◽  
Dendritic Cell ◽  
Disease Progression ◽  
Plasmacytoid Dendritic Cell ◽  
Skin Lesions ◽  
World Health ◽  
Cell Neoplasm ◽  
Blast Cells

Abstract Abstract 4703 The World Health Organization (WHO) recently published a revised, updated edition of the WHO Classification of Tumours of the Haematopoietic and Lymphoid Tissues, including new criteria for the recognition of some previously described neoplasms as well as clarification and refinement of the defining criteria for others. It also adds entities – some defined mainly by genetic and immunophenotypic features – that have only recently been characterized. Particularly, the diagnosis and classification of acute leukemias of ambiguous lineage is debated; among these: “blastic NK-cell lymphoma” and “agranular CD4+/CD56+ hematodermic neoplasms”. Both of them are now known to be, in virtually all cases, a tumor derived from precursors of a specialized subset of dendritic cells, plasmacytoid dendritic cells, and so are myeloid-related neoplasms defined as blastic plasmacytoid dendritic cell neoplasm (BPDCN). This is a clinically aggressive neoplasm that is usually characterized at onset by solitary or multiple skin lesions, often with associated regional lymphadenopathy, and frequently by involvement of the PB and BM. Leukemic cells show submembranous cytoplasmic vacuoles and pseudopodia-like extensions of agranular cytoplasm. The blasts in such cases do not express myeloperoxidase or nonspecific esterase, and are characterized by the expression of CD4, CD43, CD56, CD123, BDCA-2/CD303, TCL1, and CLA; CD7 and CD33 are not uncommonly expressed as well, and TdT is expressed in about 30% of cases. There is no expression of CD34 or CD117. Here we report three cases with clinical data, cytological and immunophenotypic findings strongly suggesting the diagnosis of BPDCN. Case 1 An 80 year-old-man was admitted to our institution on December 2006. He referred the occurrence of skin lesions since January 2005, when a diagnosis of extranodal B-cell non-Hodgkin lymphoma was made and treatment with conventional chemotherapy was performed, but without achieving any response. At our evaluation he presented leukocytosis (144 × 109/L) associated with purplish, firm nodules on the trunk, arms and face. Peripheral blood and bone marrow aspirate showed the presence of blast cells with a lymphoid appearance, granular periodic acid-Schiff (PAS) positivity and a high expression of CD33, CD4, and CD56. He was treated with AML-like therapy, but died of disease progression. Case 2 A 79-year old woman was admitted in December 2006 with a 2-month history of anemia, splenomegaly, and weight loss of 10 kg in the last year. Laboratory tests were as follows: Hb, 41 g/L; leukocytes, 2.5 × 109/L (with 10% of blast cells); platelets, 43 × 109/L. No lymphadenopathy or skin lesions were present. Bone marrow examination revealed 41% of small to medium-sized blast cells without Auer rods or granula and negative reactivity to myeloperoxidase, esterase and PAS. She was treated with an AML-like protocol; she achieved partial response, but died after three months, of disease progression. Case 3 A 69-year-old man was admitted to our Institution for cytopenia in June 2009. He referred the occurrence of brownish-purple firm nodules on the trunk since April 2009. At our evaluation he presented pancytopenia; bone marrow aspiration was performed and revealed infiltration by 65% of blasts with reticulated chromatin, evident nucleoli, a vacuolated cytoplasm and pseudopodia-like expansions. The blasts were negative for myeloperoxidase, monocyte esterase and PAS staining. Skin biopsy revealed a dermal infiltration by the same blastic-cell BM population. He underwent AML-like therapy and, although the skin lesions disappeared, 30% blastic bone marrow infiltration persisted. Morphological revision of these cases, selected for their peculiar immunophenotype reported in the following Table, revealed the same cytological features and cytochemical reactivity in cases 2 and 3; case 1 had a lymphoblastic-like morphology and showed PAS positivity, but the lack of cCD3 was not consistent with the diagnosis of ALL. All the cases were FLT3-ITD+. We suggest that a correct modern panel of MoAb with a careful morphological examination could help to pose the diagnosis of BPDCN, which typically affects older patients and is characterized by poor prognosis. Disclosures: No relevant conflicts of interest to declare.

scholarly journals A misdiagnosed case of blastic plasmacytoid dendritic cell neoplasm experiencing multiple recurrences who underwent allogeneic stem cell transplantation: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Fateme Salemi ◽  
Seyed Mohammad Reza Mortazavizadeh ◽  
Seyyedmohammadsadeq Mirmoeeni ◽  
Amirhossein Azari Jafari ◽  
Farid Kosari ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Bone Marrow Transplant ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Cervical Lymphadenopathy ◽  
Marrow Transplant ◽  
Dismal Prognosis ◽  
Cell Neoplasm ◽  
Risk Of Cancer

Abstract Background Blastic plasmacytoid dendritic cell neoplasm represents a rare type of hematologic malignancy that often manifests itself through various skin lesions. It commonly affects the elderly male population. Lymph nodes, peripheral blood, and bone marrow involvement are the typical findings that justify its aggressive nature and dismal prognosis. On histopathological assessment, malignant cells share some similarities with blastic cells from the myeloid lineage that make immunohistochemistry staining mandatory for blastic plasmacytoid dendritic cell neoplasm diagnosis. Case presentation A 35-year-old Asian man presented with cervical lymphadenopathy followed by an erythematous lesion on his left upper back. At first, the lesion was misdiagnosed as an infectious disease and made the patient receive two ineffective courses of azithromycin and clarithromycin. Six months later, besides persistent skin manifestations, he felt a cervical mass, which was misdiagnosed as follicular center cell lymphoma. Tumor recurrence following the chemoradiation questioned the diagnosis, and further pathologic assessments confirmed blastic plasmacytoid dendritic cell neoplasm. The second recurrence occurred 3 months after chemotherapy. Eventually, he received a bone marrow transplant after complete remission. However, the patient expired 3 months after transplant owing to the third recurrence and gastrointestinal graft versus host disease complications. Conclusions Early clinical suspicion and true pathologic diagnosis play a crucial role in patients’ prognosis. Moreover, allogenic bone marrow transplant should be performed with more caution in aggressive forms of blastic plasmacytoid dendritic cell neoplasm because of transplant side effects and high risk of cancer recurrence.

CD4(+)/CD56(+) Hematodermic (plasmacytoid dendritic cell) Tumor: a Highly Proliferating Neoplasm with Aberrant Expression of Immunohistochemical Markers and Poor Outcome.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2930-2930
Author(s):  
Marina Siakantaris ◽  
George Rassidakis ◽  
Flora Kontopidou ◽  
Aglaia Dimitrakopoulou ◽  
Theodoros Vassilakopoulos ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Prognostic Markers ◽  
Conflicts Of Interest ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Cell Tumor ◽  
Aberrant Expression ◽  
Cutaneous Lymphomas ◽  
The Impact

Abstract Abstract 2930 Poster Board II-906 Introduction: CD4(+)/CD56(+) hematodermic (plasmacytoid dendritic cell) tumor is a rare, distinct clinico-pathologic entity according to the latest WHO/EORTC classification for cutaneous lymphomas. Skin is typically involved at presentation followed by lymph node and bone marrow involvement and often terminating in a leukemic phase. There is no standard treatment and the prognosis remains poor. Also, there is need for identification of prognostic markers, which will better stratify these patients. Recent data show that CD4(+)/CD56(+) hematodermic neoplasms express precursor dendritic cell related antigens such as CD123, BDCA-2, and TCL1 and CLA. Patients: Ten patients (8 men, 2 women) with a median age of 72.5 years (31-86) were included in the study. They presented with cutaneous lesions plaques, nodules or tumors. Three patients had also lymphadenopathy, 3 blood leukemic picture and 4 bone marrow involvement. Results: Histological features were consistent with medium sized blastic cell lymphoma. Blastic cells were positive for CD4, CD56 and CD43. All other T-cell, B-cell and myelomonocytic markers were negative. Flow cytometry immunophenotyping was performed in skin lesions from 2 patients and in blood and bone marrow from 2 affected patients which showed a CD4+CD56+CD123bright+HLA-DRbright+CD85kbright+ phenotype. Additional immunohistochemical studies were performed on biopsy specimens using CD68, CD99 and TdT markers as well the cell proliferation marker Ki67 and the results are shown in Table 1. All patients were treated with systemic chemotherapy except one who refused treatment and died 5 months after diagnosis. Six patients achieved complete remission (CR) and 1 had partial response (PR). Five initially responding patients relapsed after a median time of 10 monhts (9-30). One patient died in CR due to treatment toxicity. Relapsed patients received salvage chemotherapy regimens. Two patents are currently alive, one in CR and one with disease. Of note, patients with TdT+ tumors showed shorter median failure-free (11 vs. 19 months) and overall (13.5 vs. 20.5 months) survival as compared to patients with TdT- neoplasms. In summary, CD4/CD56 hematodermic tumors are clinically aggressive, highly proliferating neoplasms and conventional chemotherapy used often results in a complete response but quick relapses unresponsive to further treatment may be expected. The impact of TdT expression as a potential adverse prognostic factor should be explored in larger cohort of patients. The identification of definitive prognostic markers is mandatory for the selection of patients who can benefit from bone marrow transplantation. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Blastic Plasmacytoid Dendritic Cell Neoplasm: A Rapidly Progressive and Fatal Disease without Aggressive Intervention

2013 ◽  
Vol 6 ◽  
pp. CCRep.S12608 ◽  
Author(s):  
Lindsey Prochaska ◽  
Christopher Dakhil ◽  
Sharad Mathur
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Cutaneous Manifestations ◽  
High Relapse Rate ◽  
Myeloid Neoplasm ◽  
Cell Neoplasm ◽  
Cluster Of Differentiation

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive myeloid neoplasm derived from plasmacytoid monocytes. The most common presentation involves cutaneous manifestations, which are often accompanied by bone marrow involvement. The tumor cells reveal an immature blastic appearance and diagnosis is based on the expression of cluster of differentiation (CD)4 and CD56. The literature reports a high relapse rate and poor prognosis when treated with leukemia-type induction chemotherapy alone; however, long-term remission is attainable with allogeneic stem cell transplantation in the first complete remission. Here, we report the dismal course of a patient with BPDCN with cutaneous and bone marrow involvement unable to undergo an aggressive intervention.

Blastic Plasmacytoid Dendritic Cell Neoplasm: A Clinicopathologic Review

2014 ◽  
Vol 138 (4) ◽  
pp. 564-569 ◽  
Author(s):  
Yang Shi ◽  
Endi Wang
Keyword(s):  
Acute Myeloid Leukemia ◽  
T Cell ◽  
Dendritic Cell ◽  
Myeloid Leukemia ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
World Health ◽  
Cell Transplant ◽  
Cell Neoplasm ◽  
Acute Myeloid

Blastic plasmacytoid dendritic cell neoplasm is a rare entity grouped with the acute myeloid leukemia–related precursor neoplasms in the 2008 World Health Organization classification. It was previously postulated to originate from natural killer cells, T cells, or monocytes but is now believed to arise from the plasmacytoid dendritic cell. The pathogenesis of blastic plasmacytoid dendritic cell neoplasm is not well understood, although the neoplasm demonstrates frequent deletion of tumor suppressor genes, including RB1, CDKN1B, CDKN2A, and TP53. Blastic plasmacytoid dendritic cell neoplasm is a clinically aggressive tumor that often initially presents as cutaneous lesions and subsequently progresses to bone marrow involvement and leukemic dissemination. It is characterized by enhanced expression of CD56, CD4, and CD123, which can be detected by flow cytometry/immunohistochemistry. The differential diagnoses include myeloid sarcoma/acute myeloid leukemia, T-cell lymphoblastic leukemia/lymphoma, NK-cell lymphoma/leukemia, and some mature T-cell lymphomas/leukemias. Patients usually respond to initial chemotherapy but often relapse. Stem cell transplant may improve survival.

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