[INTRODUCTION - Accumulating evidences suggest that non-T, non-B cell CD4+/CD56+ neoplasms with lymphoblastic morphology include clinically and immunophenotypically diverse entities. Although their cells of origin or classification are still controversial several entities clearly represent a distinct type of neoplasms that are clinically aggressive. CASE REPORT - In this work we present the immunophenotypic and genotypic features of bone marrow, peripheral blood, lymph node and skin lymphocytes from a patient diagnosed as plasmacytoid dendritic cell leukemia involving the skin, bone marrow, peripheral blood, lymph nodes, liver and spleen. For determination of immunophenotypic characteristics of malignant plasmacytoid dendritic cells 73 monoclonal antibodies detecting lineage markers, chemokine receptors, cytokine receptors, activation and co-stimulatory molecules were used. The malignant cells proved to express CD4+, CD56+ lineage negative leukemia phenotype characteristically positive for CD36, CD38, CD40, CD45, CD45RA, CD68, CD123, CD184, HLA-DR, BDCA2 and granzyme-B corresponding to the preplasmacitoid dendritic cell developmental stage. CONCLUSION - The presence of CD11a/CD18, CD84, CD91, CD95, αvβ5, CDw197 and the absence of CD52 and CD133 in this case can be regarded as additional features of malignant cells.]
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