A case report on BPDCN: Blastic Plasmacytoid Dendritic Cell Neoplasm
1 APGDCR, Clinical Research, Visakhapatnam, Andhra Pradesh, India.
2 APGDCR, IPCCR (NIH), Clinical Research, Visakhapatnam, Andhra Pradesh, India.
Case Study
World Journal of Advanced Research and Reviews, 2023, 19(02), 091–098
Publication history:
Received on 12 June 2023; revised on 26 July 2023; accepted on 28 July 2023
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.
Keywords:
Plasmacytoid dendritic cells (pDCs); Acute myeloid leukemia (AML); Flow cytometry; Chemotherapy; Mutation; Bone marrow
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