Blastic plasmacytoid dendritic cell neoplasm: Report of two pediatric cases.
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Date
2015-01
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Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of
acute leukemia that typically follows a highly aggressive clinical course in
adults, whereas experience in children with this disease is very limited. We
report cases of two children in whom bone marrow showed infi ltration by large
atypical monocytoid ‘blast-like’ cells which on immunophenotyping expressed
CD4, CD56, HLA-DR and CD33 while were negative for CD34 other T-cell, B-cell
and myeloid markers. The differential diagnoses considered were AML, T/NKcell
leukemia and acute undifferentiated leukemia. Additional markers CD303/
BDCA-2 and CD123 which are recently validated plasmacytoid dendritic cell
markers were done which helped us clinch the diagnosis of this rare neoplasm. An
accurate diagnosis of BPDCN is essential in order to provide prompt treatment.
Due to its rarity and only recent recognition as a distinct clinicopathological
entity, no standardized therapeutic approach has been established for BPDCN.
Description
Keywords
Blastic plasmacytoid dendritic cell neoplasm, CD123, CD303, BDCA-2, pediatric
Citation
Dharmani Preeti Ashok, Mittal Neha Manish, Subramanian P G, Galani Komal, Badrinath Yajamanam, Amare Pratibha, Gujral Sumeet. Blastic plasmacytoid dendritic cell neoplasm: Report of two pediatric cases. Indian journal of pathology& Microbiology 2015 Jan-Mar 58: (1) 72-76.