Fast Five Quiz: Blastic Plasmacytoid Dendritic Cell Neoplasm Presentation and Diagnosis

Emmanuel C. Besa, MD


February 23, 2021

Figure 1. Limb showing bruising, a symptom of lymphoid leukemia. Whereas normal white blood cells play a protective immune-response role, leukemic white blood cells are immature and abnormal, and cause decreased production of erythrocytes and platelets.

Eighty-five percent of patients with BPDCN present with skin findings, which most often are asymptomatic and non-pruritic. Dermatologic presentations are usually deep purple or brown patches, plaques, or lesions on the skin, which may be solitary, localized, or more diffuse and tend to appear primarily on the head, trunk, and/or upper extremities.

While about 30% of cases involve regional lymph nodes (secondary site), involvement of bone marrow is the more common hematologic presentation (primary site of involvement), which can manifest as thrombocytopenia, anemia or neutropenia. Despite this, patients can remain asymptomatic and bone marrow involvement is only established via CBC and additional screening. There is also a relatively high rate of CNS involvement — about 10% — upon diagnosis and in relapse.

Disease progression is usually characterized by symptoms of acute leukemia, eg, fevers, fatigue, and bruising. This is an indicator of advanced disease, with more visceral organ involvement. 

Learn more about the signs and symptoms of BPDCN.


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