Recommended Citation
Marcus D, Manera R. A Rare Case of Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm. Presented at Scientific Day; May 21, 2025; Park Ridge, IL.
Abstract
Introduction/Background:
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy that can present with or without involvement of bone marrow, skin, peripheral blood, and central nervous system. Pediatric BPDCN is less aggressive with more favorable outcomes than the more common adult BPDCN.
Description:
Our case is a 14-year-old otherwise healthy female who presented with prolonged right calf discoloration and swelling. While otherwise asymptomatic, walking and hot showers exacerbated the swelling and discoloration. Fevers, night sweats, weight loss, and bone pain were denied. The calf lesion measured a 5 x 3 cm area of presumed ecchymosis that was non-fluctuant, non-indurated, and nontender. The initial laboratory workup was unremarkable. A punch skin biopsy performed at an outside hospital was read as lymphoblastic lymphoma. On initial visit at Advocate Children’s Hospital-Oak Lawn she was noted to have a dry, non-pruritic, hyperpigmented nodular plaque measuring 13 x 12 cm. Smaller hyperpigmented lesions were present on the legs, arms, right buttock as well as a 2 x 3 cm nodular lesion on the left cheek. Repeat biopsy with flow cytometry and cytogenetics confirmed the diagnosis of BPDCN. There was no evidence of leukemic involvement. Chemotherapy utilizing the high-risk ALL protocol AALL1732 was initiated with resolution of cutaneous lesions within a month. A repeat biopsy done six months later showed no evidence of disease.
Discussion:
Pediatric BPDCN is uncommon with fewer than 100 cases reported. Clinical presentation can range from a single nodule to widespread cutaneous manifestations followed by rapid leukemic dissemination. In a case series, 73% of patients only had nodular lesions, 12% had bruise-like patches, and 14% had mixed lesions. Current treatment regimens are not standardized. Best outcomes are reported with high-risk ALL chemotherapy regimens. The role of biologic agents and stem cell transplants (SCT) are undetermined. Tagraxofusp is a biologic agent that targets CD123, a marker that is commonly overexpressed in BPDCN. Tagraxofusp has been shown in clinical trials to induce a 72% remission rate in adult patients. Additionally, both autologous and allogenic SCTs have been used successfully but are reserved for cases of second complete remission or when initial treatment does not result in rapid complete remission. Additional studies are required to further define optimal treatment in pediatric patients.
Presentation Notes
Presented at Scientific Day; May 21, 2025; Park Ridge, IL.
Full Text of Presentation
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Document Type
Poster
A Rare Case of Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm
Introduction/Background:
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy that can present with or without involvement of bone marrow, skin, peripheral blood, and central nervous system. Pediatric BPDCN is less aggressive with more favorable outcomes than the more common adult BPDCN.
Description:
Our case is a 14-year-old otherwise healthy female who presented with prolonged right calf discoloration and swelling. While otherwise asymptomatic, walking and hot showers exacerbated the swelling and discoloration. Fevers, night sweats, weight loss, and bone pain were denied. The calf lesion measured a 5 x 3 cm area of presumed ecchymosis that was non-fluctuant, non-indurated, and nontender. The initial laboratory workup was unremarkable. A punch skin biopsy performed at an outside hospital was read as lymphoblastic lymphoma. On initial visit at Advocate Children’s Hospital-Oak Lawn she was noted to have a dry, non-pruritic, hyperpigmented nodular plaque measuring 13 x 12 cm. Smaller hyperpigmented lesions were present on the legs, arms, right buttock as well as a 2 x 3 cm nodular lesion on the left cheek. Repeat biopsy with flow cytometry and cytogenetics confirmed the diagnosis of BPDCN. There was no evidence of leukemic involvement. Chemotherapy utilizing the high-risk ALL protocol AALL1732 was initiated with resolution of cutaneous lesions within a month. A repeat biopsy done six months later showed no evidence of disease.
Discussion:
Pediatric BPDCN is uncommon with fewer than 100 cases reported. Clinical presentation can range from a single nodule to widespread cutaneous manifestations followed by rapid leukemic dissemination. In a case series, 73% of patients only had nodular lesions, 12% had bruise-like patches, and 14% had mixed lesions. Current treatment regimens are not standardized. Best outcomes are reported with high-risk ALL chemotherapy regimens. The role of biologic agents and stem cell transplants (SCT) are undetermined. Tagraxofusp is a biologic agent that targets CD123, a marker that is commonly overexpressed in BPDCN. Tagraxofusp has been shown in clinical trials to induce a 72% remission rate in adult patients. Additionally, both autologous and allogenic SCTs have been used successfully but are reserved for cases of second complete remission or when initial treatment does not result in rapid complete remission. Additional studies are required to further define optimal treatment in pediatric patients.
Affiliations
Advocate Children's Hospital