Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity.
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Date
2015-07
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Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive
neoplasm classifi ed under “acute myeloid leukemia (AML) and related precursor
neoplasm” by current WHO classifi cation. Elderly male are commonly affected
with cutaneous lesion being the hallmark of disease presentation. The disease
progresses rapidly and sooner or later involves bone marrow and peripheral
blood. Cases presenting primarily as leukemia without cutaneous involvement
is a rarity with about 29 cases reported in literature till date. Characteristic
immunophenotype of CD4+/CD56+/− cells expressing antigens associated with
plasmacytoid dendritic cells like CD123, TCL1, BDCA2/CD303, cutaneous
lymphocyte-associated and interferon dependent molecule MxA, in absence
of any other lineage specifi c marker confi rms the diagnosis. The disease has a
poor survival and no standardized therapeutic strategy in the current scenario.
A case of 25-year-male presenting with leukemic BPDCN without cutaneous
involvement is presented here, who was treated with AML like protocol followed
by hematopoietic stem cell transplantation, but succumbed to the disease within
8 months of diagnosis. The present case is being fi rst to be reported from India.
Description
Keywords
Blastic plasmacytoid dendritic cell neoplasm, flow cytometry, plasmacytoid dendritic cell leukemia
Citation
Qayoom Sumaira, Durga Garima, George Seena, Rahman Khaliqur. Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous involvement in a 25 years male patient: Unusual presentation of a rare entity. Indian Journal of Pathology & Microbiology. 2015 Jul-Sept 58(3): 377-380.