Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission

Male, Heather; Davis, ML; McGuirk, Joseph P.; Abhyankar, Sunil; Aljitawi, Omar S.; Zhang, D.; Ganguly, Siddhartha

doi:10.1007/s12185-010-0655-0

Cited by 20 publications

(19 citation statements)

References 3 publications

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“…Unfortunately, transplantation beyond CR1 may have an adverse impact on both OS and progression-free survival (PFS) [42][43][44] ; thus, timing and patient selection remain important concerns. In their summary of the literature from 1994 to 2002, Reimer et al 39 reported on 10 patients: 6 patients treated with allo-SCT and 4 patients treated with autologous SCT (auto-SCT).…”

Section: Hematopoietic Sctmentioning

confidence: 99%

Treatment of blastic plasmacytoid dendritic cell neoplasm

Sullivan

Rizzieri

2016

Hematology

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare myeloid malignancy with no defined standard of care. BPDCN presents most commonly with skin lesions with or without extramedullary organ involvement before leukemic dissemination. As a result of its clinical ambiguity, differentiating BPDCN from benign skin lesions or those of acute myeloid leukemia with leukemia cutis is challenging. BPDCN is most easily defined by the phenotype CD4 1 CD56 1 CD1231 lineage -MPO -, although many patients will present with variable expression of CD4, CD56, or alternate plasmacytoid markers, which compounds the difficulty in differentiating BPDCN from other myeloid or lymphoid malignancies. Chromosomal aberrations are frequent, and the mutational landscape of BPDCN is being rapidly characterized although no obvious molecular target for chemoimmunotherapy has been identified. Chemotherapy regimens developed for acute myeloid leukemia, acute lymphoid leukemia, and myelodysplastic syndrome have all been used to treat BPDCN. Relapse is frequent, and overall survival is quite poor. Allogeneic transplantation offers a chance at prolonged remission and possible cure for those who are eligible; unfortunately, relapse remains high ranging from 30% to 40%. Novel therapies such as SL-401, a diphtheria toxin conjugated to interleukin-3 (IL-3) is commonly overexpressed in BPDCN and other aggressive myeloid malignancies and has shown considerable promise in ongoing clinical trials. Future work with SL-401 will define its place in treating relapsed or refractory disease as well as its role as a first-line therapy or bridge to transplantation. Learning Objectives• To understand the clinical and laboratory approach for differentiating BPDCN from acute myeloid leukemia with cutaneous manifestations • To consider appropriate first-line and salvage treatments for BPDCN, including the utility of hematopoietic allogeneic stem cell transplantation and novel agents in various patient populations

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Section: Hematopoietic Sctmentioning

confidence: 99%

Treatment of blastic plasmacytoid dendritic cell neoplasm

Sullivan

Rizzieri

2016

Hematology

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“…Actualmente se considera la mejor opción el TAloCPH durante la primera remisión completa, aumentando la supervivencia global y supervivencia libre de enfermedad, sin recaídas dentro de los primeros 27 meses 18,19 . En nuestros pacientes el tratamiento consistió en HiperCVAD seguido de TAloCPH con una media de seguimiento de 14,6 meses.…”

Section: Discussionunclassified

Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

et al. 2017

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Blastic plasmacytoid dendritic cell neoplasm.Report of three cases L a neoplasia blástica de células dendríticas plasmocitoides (NBCDP) es una entidad relativamente rara que afecta principalmente pacientes adultos del sexo masculino 1,2 . Su contraparte normal es la célula dendrítica plasmocitoide de origen hematopoyético, la cual expresa el receptor α de la IL-3 (CD123) en su superficie y carece de marcadores de estirpe mieloide y linfoide. Su función biológica consiste principalmente en establecer un mecanismo de regulación entre la respuesta inmunológica innata y específica 3,4 .La mayoría de los casos se manifiestan como una enfermedad cutánea en forma de nódulos y/o placas 5 , con compromiso de la médula ósea y/o sangre periférica 6 .El diagnóstico se establece mediante el estudio histopatológico de las lesiones cutáneas, caracterizado por la proliferación de células neoplásicas de aspecto blástico que infiltran la dermis y en algunos casos el tejido subcutáneo, con expresión de CD4, CD56, CD123, TCL-1 y CD303 4 . El comportamiento biológico es agresivo y muchos pacientes fallecen en un período corto después del diagnóstico 3 .El objetivo de este estudio es informar las características clínico-patológicas de la NBCDP diagnosticados en el Instituto Nacional de Cancerología de México.

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“…However, BPDCN widely disseminates, and despite an initial response to systemic chemotherapy, relapses are frequent in the absence of allogeneic hematopoietic stem cell transplantation (allo-SCT). Treatment with acute myeloid leukemia (AML)-type induction chemotherapy regimens followed by allo-SCT at the first complete remission has been advocated (4,5). Although the older age of the patient populations presents a challenge to the treating hematologist/oncologist, reduced-intensity conditioning allo-SCT has been shown to be effective in patients up to 70 years of age (6).…”

Section: Discussionmentioning

confidence: 99%

Case study interpretation—New Orleans: Case 5

Roth

Robinson

2013

Cytometry Part B Clinical

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CASE HISTORYThe patient is an 81-year-old gentleman who was referred to the hematology/oncology service for worsening fatigue and skin lesions. His past medical history is significant for myelodysplastic syndrome diagnosed 5 months ago. He was symptomatically treated and followed a relatively stable course until approximately 1 month ago. At that time, he noticed multiple skin lesions over his trunk and legs, and also became increasingly fatigued with exertional dyspnea, finding himself short of breath after climbing one flight of stairs. Laboratory data were significant for 30% circulating blasts, neutropenia (ANC ¼ 0.96/mm 3 ), macrocytic anemia (Hgb 7.7 g/dL, MCV 109.6), and thrombocytopenia (52 Â

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Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission

Cited by 20 publications

References 3 publications

Treatment of blastic plasmacytoid dendritic cell neoplasm

Treatment of blastic plasmacytoid dendritic cell neoplasm

Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

Case study interpretation—New Orleans: Case 5

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