Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review

Rauh, Michael J.; Rahman, Fazlur; Good, David; Silverman, Jeffrey A.; Brennan, Michael K.; Dimov, Nikolay; Liesveld, Jane L.; Ryan, Daniel; Burack, W. Richard; Bennett, John M.

doi:10.1016/j.leukres.2011.07.033

Cited by 85 publications

(69 citation statements)

References 20 publications

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“…Isolated skin lesions are frequently the first symptom leading patients to seek medical advice, and without therapy, they disseminate rapidly. Conversely BPDCN without cutaneous involvement at onset is rarely reported: 20,21 in our study 23% of patients had no cutaneous manifestations at diagnosis, and among them only two developed skin lesions during progression of the disease. We found frequent neuromeningeal involvement (16%), both at diagnosis and at relapse, as in other studies in which the incidence of CNS disease ranged from 9% to 26%.…”

Section: Discussioncontrasting

confidence: 49%

Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

Pagano

Valentini

Pulsoni³

et al. 2012

Haematologica

288

365

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The objective of this study was to evaluate the clinical features, prognostic factors, and efficacy of treatments in patients with blastic plasmacytoid dendritic cell neoplasm with a leukemic presentation at onset of the disease. In order to do this, a retrospective multicenter study was performed from 2005-2011 in 28 Italian hematology divisions in which 43 cases were collected. Forty-one patients received an induction therapy, consisting of an acute myeloid leukemia-type regimen in 26 patients (60%) and acute lymphoid leukemia/lymphoma-type regimen in 15 patients (35%). Six patients (14%) underwent allogeneic hematopoietic stem cell transplantation. Seventeen patients (41%) achieved a complete remission: seven after acute myeloid leukemia-type treatment and 10 after an acute lymphoid leukemia/lymphoma-type regimen, with a significant advantage for acute lymphoid leukemia/lymphoma-type chemotherapy (P=0.02). Relapse occurred in six of the 17 patients (35%) who achieved complete remission, more frequently after acute lymphoid leukemia/lymphoma-type chemotherapy. The median overall survival was 8.7 months (range, 0.2-32.9). The patients treated with an acute myeloid leukemia-type regimen had an overall survival of 7.1 months (range, 0.2-19.5), whereas that of the patients receiving acute lymphoid leukemia/lymphoma-type chemotherapy was 12.3 months (range, 1-32.9) (P=0.02). The median overall survival of the allogeneic hematopoietic stem cell transplant recipients was 22.7 months (range, 12-32.9), and these patients had a significant survival advantage compared to the non-transplanted patients (median 7.1 months, 0.2-21.3; P=0.03). In conclusion, blastic plasmacytoid dendritic cell neoplasm with bone-marrow involvement is an aggressive subtype of high-risk acute leukemia. The rarity of this disease does not enable prospective clinical trials to identify the better therapeutic strategy, which, at present, is based on clinicians' experience. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

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Section: Discussioncontrasting

confidence: 49%

Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

Pagano

Valentini

Pulsoni³

et al. 2012

Haematologica

288

365

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“…However, pediatric cases, even in infants and young children, have also been reported (14,15). Clinically, there is nearly always multiple organ involvement, and cutaneous involvement characterized with tumor cells located under the dermis is the main initial presentation of BPDCN in the majority of the cases, although occasionally there is no cutaneous involvement and the disease presents with leukemia symptoms (16,17); in addition, extracutaneous involvement often occurs simultaneously, such as in the lymph nodes, peripheral blood, bone marrow, skeleton, gastrointestinal tract, central nervous system, lung, mediastinum and pancreas (18)(19)(20)(21); however, to the best of our knowledge, testicular involvement has not been reported to date. The patient in case 1 exhibited typical clinical manifestations, with initial skin involvement, followed by rapid occurrence of multiple organ involvement (lymph nodes, liver, spleen and bone marrow); as regards the second patient, there was simultaneous occurrence of bilateral maxillofacial involvement, confirmed by histopathological examination of a surgical biopsy specimen, bilateral testicular involvement and mediastinal involvement, confirmed by histopathological examination of a needle biopsy specimen, prior to bone marrow involvement.…”

Section: Discussionmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm in children: A review of two cases

Deng

Yang

Kuang

et al. 2017

Molecular and Clinical Oncology

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Abstract. Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly characterized, rare malignant tumor of the skin and hematopoietic system. BPDCN occurs mainly in the elderly, whereas it is rarer among children, and has variable clinical manifestations. Optimal chemotherapeutic regimens for the treatment of BPDCN have not yet been determined and this tumor has a poor prognosis. In this study, two pediatric cases of BPDCN, including a 7-year-old female and a 9-year-old male patient, diagnosed at the Xiangya Hospital of Central South University over the past 2 years, were retrospectively reviewed. Both cases exhibited multiple organ involvement, although the clinical manifestations differed; they were diagnosed with BPDCN based on the clinical manifestations, pathological and immunohistochemical findings, which included positivity for CD4, CD56 and CD123. A high-risk acute lymphocytic leukemia (ALL) chemotherapy regimen was administered to both patients. The patient in the first case achieved a complete remission, but unfortunately her parents refused follow-up treatment and she succumbed to the disease 9 months after the initial diagnosis. The second patient was treated for a total of three courses with a chemotherapy regimen including daunorubicin, cytarabine and etoposide, followed by two courses of the high-risk ALL chemotherapy regimen; unfortunately, a remission was not achieved and the patient was scheduled to receive hematopoietic stem cell transplantation. Thus, not all pediatric BPDCN patients may be able to achieve complete remission following chemotherapy with the high-risk ALL regimen, and other treatment options must be investigated in the future.

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“…[14][15][16] Cutaneous involvement (either in the form of isolated purple nodules or macules or disseminated lesions) is estimated to occur in 85% of patients. 2 Central nervous system involvement is estimated to occur in 9% to 26% of patients. [17][18][19] In the current series, central nervous system involvement was more frequent (4 of 5 patients).…”

Section: Discussionmentioning

confidence: 99%

“…1 It is clinically characterized by frequent (85%) cutaneous involvement. 2 It is a highly malignant disease with a poor prognosis; a mean survival of 12 months was observed in what to our knowledge is the largest series published to date. 3 Due to its highly malignant behavior, a fast and correct diagnosis of this condition is of the utmost importance.…”

Section: Introductionmentioning

confidence: 93%

Cytomorphological features of blastic plasmacytoid dendritic cell neoplasm on FNA and cerebrospinal fluid cytology: A review of 6 cases

Ferreira¹,

Gasparinho

Fonseca³

2015

Cancer Cytopathology

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BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic tumor that was once believed to be derived from natural killer cells and is now recognized as originating from precursors of plasmacytoid dendritic cells. It generally involves the skin and has an aggressive clinical course. Due to its highly malignant behavior, a fast and accurate diagnosis of this condition is of the utmost importance. METHODS: Six cytology specimens from 5 patients diagnosed with BPDCN were reviewed as well as their clinical records. Findings were compared with 3 previously published cases. RESULTS: Two exfoliative cytology specimens (cerebrospinal fluid) and 4 fine-needle aspiration specimens (3 lymph node specimens and 1 cutaneous specimen) were reviewed. The cytomorphological aspects were similar in all cases. The smears were hypercellular with a monotonous population of intermediate-sized cells, dispersed singly or arranged in loose aggregates. The cells had round to oval nuclei, with fine chromatin and prominent nucleoli; the cytoplasm was generally scant, without visible granules. Intracytoplasmic microvacuoles were found in the majority of cases.Four cases were also characterized by flow cytometry, which revealed expression of CD 123, CD56 and/or CD4. Fineneedle aspiration was used in 1 case for primary diagnosis. Histological confirmation was available in all cases. CONCLU-SIONS: BPDCN is a highly malignant neoplasm with a poor outcome. Cytology, in association with flow cytometry immunophenotyping and clinical history, is a reliable method with which to establish the diagnosis. Cancer Cytopathol 2016;124:196-202.

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Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review

Cited by 85 publications

References 20 publications

Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

Blastic plasmacytoid dendritic cell neoplasm in children: A review of two cases

Cytomorphological features of blastic plasmacytoid dendritic cell neoplasm on FNA and cerebrospinal fluid cytology: A review of 6 cases

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