Blastic plasmacytoid dendritic cell neoplasm: a rare lymphoma of extremely aggressive course

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as blastic NK cell lymphoma or CD4+/CD56+ hematodermic neoplasm, is a rare aggressive disorder of a not fully understood etiology [1]. It predominantly involves the skin and has a high risk of leukemic dissemination [2]. The disease has an aggressive course and poor long-term prognosis with a median survival of 12–16 months [3]. We report a patient, who displayed clinical and immunohistochemical features of BPDCN with no primary bone marrow involvement. The disease had an extremely rapid course, which led to the patient’s death in 4 months after the first lesions had developed.
A 70-year-old Caucasian male was referred to our Department in December 2014 with a 2-month history of rapidly developing asymptomatic nodules and plaques on the head, neck and upper part of the trunk. On physical examination, we saw disseminated red-to-purple indurated nodules and bruise-like plaques located predominantly within the head, neck and upper part of the trunk (Figure 1). The biggest plaques, located on the right cheek, chin and back were round, well-circumscribed, red-to-purple, had up to 7 cm in diameter and presented discrete scaling on the surface. Numerous smaller red and brownish indurated lesions were located within the face, back and chest. There were no signs of peripheral lymphadenopathy. Basic laboratory work-up revealed leucopenia (WBCs 3.07 × 109/l) with thrombocytopenia (PLTs 102 × 109/l) and elevated levels of serum creatinine (1.5 mg/dl) and 2-microglobulin (6 mg/l). The chest X-ray was normal, while ultrasonography displayed hepatosplenomegaly and slight enlargement of the axillary lymph nodes. A 5-mm punch biopsy was performed. On histology, the specimen showed dense dermal and subcutaneous aggregates of small-to-medium sized pleomorphic T-cells with no features of epidermotropism. Immunohistochemically, the infiltration was CD3+/–, CD4–/+, CD5–/+, CD7–, CD8–/+, CD20–, CD30–, CD56+, CD123+ (Figure 2). The Ki-67 proliferation rate was 30–35%. The infiltration was negative for Ebstein-Barr virus. Bone marrow biopsy was within normal limits. Taking into consideration rapid progression of cutaneous lesions, the patient was qualified for systemic chemotherapy. However, during the second hospitalization, before chemotherapy was initiated, the number of white blood cells, red blood cells and platelets decreased significantly (WBCs 2.19 × 109/l, RBCs 2.72 × 1012/l, PLTs 69 × 109/l)....


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