(Last Updated On: October 30, 2018)

Authors: Jennica Celine S. Ponce, MD; Ma. Flordeliz A. Casintahan, MD, FPDS; Margaret Mary B. Alegre, MD, DPDS

Abstract

Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematodermic malignancy that usually occurs in elderly individuals and manifests with skin lesions followed by involvement of lymph nodes, bone marrow, and peripheral blood. There is a high risk for leukemic dissemination, hence, a poor prognosis.

Case Summary: A case of a 43-year old male is presented, with a 5-month history of solitary, well-circumscribed, non-movable, non-tender, 5.0 x 5.0 cm brown mass with overlying brownish-red papules, and surrounding erythematous plaque with follicular accentuation on the right upper back; and multiple erythematous patches and plaques on face, trunk, and upper extremities, with accompanying occasional easy fatigability, cervical lymphadenopathies, and pancytopenia. Histopathologic examination showed grenz zone with overlying flat epidermis, and nodular diffuse dermal infiltrates of atypical lymphocytes with hyperchromatic nuclei and prominent chromatin. Atypical cells extend around blood vessels and arrector pili muscle with atypical mitotic figures. Immunohistochemical profile showed positivity for CD56, CD4, Bcl-2, Ki-67, and S100; and negativity for CD3, CD20, CK20, CD45RO, CD99, CD10, and CD138. All of which were consistent with BPDCN.

Conclusion: A diagnosis of BPDCN is typically determined based on the histopathological and immunohistochemical examinations. However, despite the increasing number of cases reported in recent years, the diagnosis and treatment still remain a challenge due to the rarity of this diagnosis worldwide, as well as the clinical and phenotypical diversity observed among patients, and probable overlapping features with other hematologic malignancies. No standardized treatment regimen has been established as of this time.

 

Citation

 

Keywords

blastic plasmacytoid dendritic cell neoplasm, CD4 positive, CD56 positive,
hematocutaneous neoplasm, case report

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