Blastic Plasmacytoid Dendritic Cell Neoplasm: A Rapidly Progressive and Fatal Disease without Aggressive Intervention | Zendy

Lindsey Prochaska | Zendy; Christopher Dakhil | Zendy; Sharad C. Mathur | Zendy

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Blastic Plasmacytoid Dendritic Cell Neoplasm: A Rapidly Progressive and Fatal Disease without Aggressive Intervention

Author(s) -

Lindsey Prochaska,

Christopher Dakhil,

Sharad C. Mathur

Publication year - 2013

Publication title -

clinical medicine insights case reports

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.187

H-Index - 12

ISSN - 1179-5476

DOI - 10.4137/ccrep.s12608

Subject(s) - medicine , neoplasm , bone marrow , complete remission , myeloid , chemotherapy , myeloid leukemia , leukemia , disease , pathology , oncology , immunology

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive myeloid neoplasm derived from plasmacytoid monocytes. The most common presentation involves cutaneous manifestations, which are often accompanied by bone marrow involvement. The tumor cells reveal an immature blastic appearance and diagnosis is based on the expression of cluster of differentiation (CD)4 and CD56. The literature reports a high relapse rate and poor prognosis when treated with leukemia-type induction chemotherapy alone; however, long-term remission is attainable with allogeneic stem cell transplantation in the first complete remission. Here, we report the dismal course of a patient with BPDCN with cutaneous and bone marrow involvement unable to undergo an aggressive intervention.

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