Figure 1.
Figure 1. Morphologic evaluation and immunohistochemical markers that aid in the diagnosis of BPDCN. (A) Skin lesions of BPDCN can vary in shape, size, color, and distribution. Hyperpigmented red-brown macules, shown here, may be confused with neoplastic and nonneoplastic etiologies. (B) Skin biopsy (×500) of dermal infiltrate of immature mononuclear cells, which spares the epidermis (separated by a Grenz zone) typical of BPDCN, LC, and myeloid sarcoma (MS) and helps distinguish those diseases from mycosis fungoides which is usually epidermotropic. (C) Bone marrow aspirate (×1000) demonstrates medium to large cells with scant cytoplasm, immature chromatin, irregular nuclear contours, and prominent nucleoli. (D) Shared immunohistochemical markers are shown with a range of positive cases observed for BPDCN and AML/LC/MS. Ranges are rounded to the nearest 5% based on multiple series.2,17,18,26 Clearly the overlap of shared markers and exception of atypical cases that lack a particular marker highlight the need for review of unique markers to differentiate BPDCN from AML/LC/MS.

Morphologic evaluation and immunohistochemical markers that aid in the diagnosis of BPDCN. (A) Skin lesions of BPDCN can vary in shape, size, color, and distribution. Hyperpigmented red-brown macules, shown here, may be confused with neoplastic and nonneoplastic etiologies. (B) Skin biopsy (×500) of dermal infiltrate of immature mononuclear cells, which spares the epidermis (separated by a Grenz zone) typical of BPDCN, LC, and myeloid sarcoma (MS) and helps distinguish those diseases from mycosis fungoides which is usually epidermotropic. (C) Bone marrow aspirate (×1000) demonstrates medium to large cells with scant cytoplasm, immature chromatin, irregular nuclear contours, and prominent nucleoli. (D) Shared immunohistochemical markers are shown with a range of positive cases observed for BPDCN and AML/LC/MS. Ranges are rounded to the nearest 5% based on multiple series.2,17,18,26  Clearly the overlap of shared markers and exception of atypical cases that lack a particular marker highlight the need for review of unique markers to differentiate BPDCN from AML/LC/MS.

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