Abstract
Dendritic cells are present in both lymphoid and non-lymphoid organs, which are heterogenous group of nonphagocytic and nonlymphoid immune accessory cells. Plasmacytoid dendritic cell (which has an eccentric nuclei) are subset of dendritic cells aid in secreting high levels of type 1 interferons by activation and gaining dendritic cell like morphology, play an important role in antiviral immunity and are involved in initiation and development stages of inflammatory response. The initial presentation of extra-cutaneous region is an involvement of regional lymph nodes later progressed to bone marrow and peripheral blood. When bone marrow is extensively involved, neoplastic cells interfere peripheral blood resembling as circulating leukemic myeloid or lymphoid blasts. BPDCN is confused with cutaneous peripheral T-cell lymphoma due to cutaneous involvement. Comparative to adults, BPDCN in children are less aggressive. Intensive 1st line therapy along with “Lymphoid type” chemotherapy show better results. Lymphodenopathy, splenomegaly, bone marrow involvement and cytopenia, cutaneous lesions are observed widely.
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