Successful treatment of pediatric plasmacytoid dendritic cell tumors with a contemporary regimen for acute lymphoblastic leukemia

Wright, Karen; Onciu, Mihaela; Coustan‐Smith, Elaine; Campana, Dario; Raimondi, Susana C.; Inaba, Hiroto; Ribeiro, Raul C.; Pui, Ching‐Hon; Sandlund, John T.

doi:10.1002/pbc.24483

Cited by 8 publications

(11 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, among commercial anti-CD22 antibody clones, only s-HCL-1 appears to be capable of recognizing an epitope expressed on pDCs and BPDCN. Expression of CD22 was also reported in cases of pediatric BPDCN [19]. One of two cases assessed in our study was positive for CD22 using the s-HCL-1 clone.…”

Section: Discussionsupporting

confidence: 77%

See 1 more Smart Citation

TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow

et al. 2013

View full text Add to dashboard Cite

*Distinguishing blastic plasmacytoid dendritic cell neoplasm (BPDCN) from acute myeloid leukemia (AML) is gaining increased importance because of emerging differences in therapeutic approaches, and this distinction can be problematic in bone marrow specimens. We identified retrospectively 16 patients with bone marrow involvement by BPDCN: 11 men and 5 women with a median age of 62.5 years (range, 19-86 years). Myelodysplastic changes were observed in five patients. Immunophenotypic analysis showed that the neoplastic cells were positive for CD4, CD123, TCL-1, and HLA-DR and were negative for CD3, CD8, CD13, CD19, CD34, and myeloperoxidase. Other antigens expressed by subsets of BPDCN cases included the following: CD56 (13/15; 81%), CD33 (7/10; 70%), CD7 (11/14; 69%), TdT (5/15; 33%), CD2 (5/11; 31%), CD117 (2/ 9; 22%), and CD5 (2/13; 15%). Conventional cytogenetic analysis showed chromosomal abnormalities in 6 of 13 (46%) cases analyzed, of which 3 cases had 213/13q2. Targeted next-generation sequencing performed on five BPDCN cases identified TET2 (ten eleven translocation 2) mutations and no other AMLassociated mutations. In conclusion, BPDCN in the bone marrow has a characteristic immunoprofile (CD41, CD561, CD1231, and TCL-11) and appears to be commonly associated with myelodysplastic features and a high frequency of TET2 mutations in the absence of other mutations commonly observed in AML. Am.

show abstract

Section: Discussionsupporting

confidence: 77%

“…particularly robust and deliver intense staining reactions, a useful feature in assessing residual disease. As reported by others, other antigens expressed in variable subsets of BPDCN include CD2, CD5, CD7, CD33, and CD117 [17][18][19]. Reineks et al have identified CD22 expression in normal pDCs and in four cases of BPDCN [20].…”

Section: Discussionmentioning

confidence: 80%

TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow

et al. 2013

View full text Add to dashboard Cite

show abstract

“…These lesions vary from hematoma-like macules to purplish plaques or nodules; focal lesions often affect the head and lower extremities, although they may spread throughout the body [ 11 , 12 ]. As shown in Figure 1 , the characteristic lesions of the cutaneous involvement are non-specific and are easily confused with other differential diagnoses [ 3 , 4 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…It is derived from a subset of precursor cells called plasmacytoid dendritic cells or type II dendritic cells, characterized by the expression of CD123, CD4, and CD56 in the absence of markers of other lineages [1,2]. Although the disease predominantly affects older adults, with a median age of 65 years, it has been reported in other age groups, including infants and children [3][4][5].…”

Section: Introductionmentioning

confidence: 99%

Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm, Clinical Features and Immunophenotype: A Case Report

Suárez¹,

Soler²,

Carreño³

et al. 2023

Cureus

View full text Add to dashboard Cite

show abstract

“…BPDCN typically affects elderly patients; however, it can be present at any age [ 3 ]. Pediatric patients are exceedingly rare with an unclear clinical course [ 3 , 4 ]. Currently, no standardized therapeutic approach has been established, although an acute lymphoblastic leukemia (ALL) type of treatment appears to be more effective in those patients who are able to tolerate aggressive chemotherapy [ 2 – 4 ].…”

Section: Introductionmentioning

confidence: 99%

First pediatric experience of SL-401, a CD123-targeted therapy, in patients with blastic plasmacytoid dendritic cell neoplasm: report of three cases

et al. 2018

View full text Add to dashboard Cite

BackgroundBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly aggressive hematological malignancy with extremely poor outcome. The median overall survival for adult patients is 9–13 months. Pediatric patients are exceedingly rare with an unclear clinical course. Currently, no standardized therapy has been established, although an acute lymphoblastic leukemia type of treatment appears to be more effective in those patients who are able to tolerate aggressive chemotherapy. SL-401 is a targeted therapy directed to CD123, a protein ubiquitously expressed at high level on the surface of BPDCN blasts. In adult phase 2 trials, it has demonstrated efficacy with 90% overall response rate. No pediatric patients with BPDCN using SL-401 have been reported.Case presentationHere, we report the first pediatric experience of three children with BPDCN treated with SL-401 at our institution. All patients tolerated SL-401 without significant toxicities. One patient with multiply relapsed and refractory disease had no response. The other two cases had significant and rapid clinical improvement after the two courses of treatment. However, the response was transient, and growth of soft tissue mass was observed in-between cycles in both patients with large tumor burden.ConclusionsThis is the first report of SL-401 in pediatric patients with BPDCN. Sl-401 was well tolerated and can produce a promising response. Further testing this agent in children is warranted.

show abstract

Successful treatment of pediatric plasmacytoid dendritic cell tumors with a contemporary regimen for acute lymphoblastic leukemia

Cited by 8 publications

References 20 publications

TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow

TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow

Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm, Clinical Features and Immunophenotype: A Case Report

First pediatric experience of SL-401, a CD123-targeted therapy, in patients with blastic plasmacytoid dendritic cell neoplasm: report of three cases

Contact Info

Product

Resources

About