Journal of the Louisiana State Medical Society
Table 1: Summary of the immunophenotypic profiles of BPDCN Antibody Expression
Reference
CD2 CD3 CD4 CD5 CD7
variable, + in 33%-51%
8,9,15
-; rare cases cytoplasmic CD3+
11,18,20
+ (80%-100%)
4,6,14,15,17,21-23
-; one case +
19
-; a few cases + (subset or focal)
10,11
CD33 CD43
-; rare cases +
8,13
+ (96%)
4,6,14,15,17,21-23 4,6,14,15,17,21-23
CD45RA
+ (dim)
CD56 CD68
+ (75%-100%)
6,14,15,17,21-23
variable, + in 52%-85%, cytoplasmic dots in some cases
8-10,15
CD34
-; rare cases + -; rare cases +
4 4
CD117
TdT
variable, + in 14%-63%
9-12, 15
CD123
+ (75%-100%) + (82%-100%) + (50%-100%)
6,14,15,17,21-23 6,14,15,17,21-23 6,14,15,17,21-23 6,14,15,17,21-23 6,14,15,17,21-23 6,14,15,17,21-23
TCL1
BDCA2/CD303
CD2AP
+ (45%-95%)
BCL11A
+ (83%-100%) + (90%-100%)
CLA
CD19, CD20, CD22, CD79a, PAX5
- -
4,7-10 4,7-10
EBER
MPO, Lysozyme
-
4,6,14,15,17,21-23
S100
variable, + in 75% children, + in 16%- 25% adults
3,8,10
Table 1: Summary of the immunophenotypic profiles of BPDCN, or blastic plasmacytoid dendritic cell neoplasm. BPDCN; BDCA2/ CD303, blood dendritic cell antigen 2; TCL1, T-cell leukemia/lymphoma 1; CD2AP, CD2-associated protein; CLA, cutaneous lymphocyte antigen; BCL11A, B-cell lymphoma/leukemia 11A; MPO, myeloperoxidase; TdT, terminal deoxynucleotidyl transferase; EBER, EBV-encoded nuclear RNA.
during extracorporeal photopheresis for GVHD. Whether extracorporeal photopheresis stimulates malignant trans- formation of PDCs remains to be determined, but careful clinical selection of GVHD treatment might be warranted. Morphology Histopathologically, BPDCN in the skin is character- ized by a diffuse infiltrate of medium-sized blasts with high N:C ratio, scant cytoplasm, finely dispersed chromatin, and prominent nucleoli in the dermis underlying a Grenz zone without epidermotropism present. Cota et al. showed that the typical blasts were seen in 44% of skin biopsy specimens of BPDCN, with the majority (56%) of cases presenting as
a rather pleomorphic infiltrate with admixed blastoid cells in the background. 15 On bone marrow aspirate smears, the blasts have scant agranular cytoplasm with eccentrically located nucleus and perinuclear hof. 4 On cytology prepara- tion, the blasts have round to oval nuclei, finely dispersed chromatin, prominent nucleoli, scant pale-blue agranular cytoplasm, occasional cytoplasmic microvacuoles, and pseudopodia-shape cytoplasmic extension. 8,13 Immunophenotype The immunophenotypic profiles of BPDCN are sum- marized in Table 1. BPDCN characteristically expresses CD4, CD56, CD43, CD45, and PDC-associated antigens
6 J La State Med Soc VOL 166 January/February 2014
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