Clinical and Medical Case Reports

Biography

Biography: Ilina Micheva

Abstract

Blastic plasmacytoid dendritic cell leukemia (BPDCL) is a rare, clinically aggressive hematologic malignancy derived from the precursors of plasmacytoid dendritic cells and characterized by co-expression of CD4 and CD56 without other lineage-specific markers. Its misleading clinical presentation resembles many other aggressive diseases like myeloid, lymphoid, NK neoplasms and malignant melanoma.

Our patient was a 67-years-old male who presented with complains of asthenia and diffuse generalized rash of blackish palpable plagues on his back, chest and hands that have appeared 5-6 months before hospitalization.  He had no B symptoms, no lymph adenomegaly and hepatosplenomegaly. The CBC showed leukocytosis (22,93.10⁹/L), anemia (Hb-75 g/L) and thrombocytopenia (36.10⁹/L). The blood smear and bone marrow revealed 45 %  and respectively 70 % of blasts with morphological characteristics of lymphoblasts. Flow cytometry showed a CD45^low population expressing CD4, CD56, CD303, CD304, and high levels of CD123 and TCL1. There was no T-cell, B-cell or myeloid expression except for CD33 and CD2. Karyotype was normal. CT revealed  mediastinal and abdominal lymphadenomegaly and splenomegaly.Treatment according to Hyper-CVAD protocol was initiated. A week later the patient became somnolent and bradypsychic. MRT revealed multiple bilateral hyperdense lesions with perifocal edema. In two days the patient passed out.

BPDCN could be hardly distinguished from several hematological and oncological neoplasms. Despite its low incidence, clinicians should include it in differential diagnosis of tumors with cutaneous, blood, lymphatic tissue and CNS involvement.  The  early diagnosis with intensive chemotherapy and CNS prophylaxis may  improve the outcome.