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2015 ; 37
(12
): 924-8
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Blastic Plasmacytoid Dendritic Cell Neoplasm in the Pediatric Population: A Case
Series and Review of the Literature
#MMPMID26588336
Nguyen CM
; Stuart L
; Skupsky H
; Lee YS
; Tsuchiya A
; Cassarino DS
Am J Dermatopathol
2015[Dec]; 37
(12
): 924-8
PMID26588336
show ga
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive
hematologic malignancy primarily found in adults, often carrying a poor
prognosis. There are only 33 reported pediatric cases of BPDCN in the literature.
Although standard treatment is not yet established for children, current
literature recommends the use of high-risk acute lymphoblastic leukemia
(ALL)-type chemotherapy. Recent studies, however, have explored the benefits of
combining chemotherapy with stem-cell transplantation. Here, the authors present
2 cases of pediatric BPDCN treated with different modalities. The first case is a
13-year-old girl who presented with a 3-month history of an initially
asymptomatic firm nodule on her left shin. The second case is a 15-year-old boy
who presented with a 4-month history of an enlarging subcutaneous nodule on the
lower leg. Immunohistochemical staining of both patients was positive for markers
consistent with BPDCN. The latter patient received ALL-type therapy alone,
whereas the former received ALL-type chemotherapy and stem-cell transplantation.
Since initial treatment, both patients remain disease-free. These cases
contribute to the limited number of pediatric BPDCN cases, thus helping to
advance our knowledge toward an optimal treatment protocol for clinical
remission.
|Adolescent
[MESH]
|Antineoplastic Combined Chemotherapy Protocols/therapeutic use
[MESH]