Agranular CD4/CD56 Positive Hematodermic Neoplasm (CD56 Positive Non-Hodgkin Lymphoma)

CD4+/CD56+ hematodermic neoplasm is a rare type of primary cutaneous T-cell lymphoma and so far the single representative of the category precursor hematologic neoplasm. The tumor cells express CD56 and have therefore been suggested to represent a natural-killer(NK)-cell-lymphoma. The finding of CD123 positivity on the tumour cells now assures the origin from plasmacytoid dendritic cells. The tumour mostly shows a clinical course with high mortality caused by lymph node and bone marrow involvement often followed by a terminal leukemic phase. We present a patient treated with systemic chemotherapy (KNOSPE-scheme) and satisfactory results until now.