Morphologic Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive lymphoma derived from plasmacytoid dendritic cells or precursor dendritic cells. Despite some 240 reported cases, its morphology and especially ultrastructure has not been satisfactorily studied. A case is reported of a 13 year old boy, who, despite chemotherapy, died within a 12-month period. The electron microscopy findings - microvillous processes, nuclei with slight irregularities, a moderate amount of heterochromatin, and rough endoplasmic reticulum in the form of long, narrow profiles, often in parallel arrangements - taken together, serve to distinguish BPDCN from other neoplastic cells, such as monocytes, plasma cells and the cells of chronic lymphocyte leukemia.

Novel Ultrastructural Findings of Blastic Plasmacytoid Dendritic Cell Neoplasm

“…In previous studies in which TEM of bone marrow BPDCN was performed, CD34 and c-kit immunostainings were not performed, and thus the maturation stage was not clarified. [ 4 5 ] Although those cases showed a rough endoplasmic reticulum, our case also showed transport vesicles, which support the mature phenotype of BPDCN. In conclusion, the combination of immunohistochemical and ultrastructural analyses enables a more accurate diagnosis of this clinically heterogeneous tumor to be made.…”

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)

“…The lymph nodes show a diffuse interfollicular and medullary involvement. Microscopic examination of the skin biopsy typically shows a diffuse monotonous infiltrate of intermediate-sized blast cells with round to oval nuclei and nucleoli, and scant agranular cytoplasm that resemble myeloid blasts, but with NK cell lineage differentiation (Ferreira et al, 2016;Ru et al, 2014). Intracytoplasmic microvacuoles are present in the majority of cases (Ferreira et al, 2016).…”

Plasmocytoid Dendritic Cells Leukemia: A Rare Presentation

“…These cytological features are found in our case which prompted us to test specific markers of pasmacytoid dendritic cells. However, the proliferation can take different forms [10,11]. At last, negative cytochemical reactions (myeloperoxidases, esterases) and results of immunophenotyping will allow to exclude other diagnosis hypothesis [12].…”

“…Two thirds of patients with LpDC have chromosomal abnormalities [9,11,13]. In most cases, Karyotypes are complex and associate within the same clone an original combination of recurrent anomalies.…”

“…Its prognosis is poor, the median survival is less than 16 months, even for patients with pure cutaneous form. Relapses are common and early with frequent neuromeningeal attacks [2,10,11].…”