Anatomic Pathology: Hematopathology

959) Which hematopoietic malignancy has the following immunophenotype: CD4 positive, CD56 positive, CD123 positive, TCL1 positive, CD2 positive/negative, TdT positive/negative, CD3 negative, CD5 negative, CD8 negative, CD34 negative, and Epstein Barr virus (EBV) negative?

• Blastic plasmacytoid dendritic cell leukemia essentially has the same immunophenotype as normal plasmacytoid dendritic cells (CD4 positive, CD123 positive), but with the additional aberrant expression of CD56 and TdT.

Aggressive natural killer (NK)-cell leukemia (B) has the following immunophenotype: CD2 positive, surface CD3 negative, CD4 negative, CD5 negative, CD8 positive/negative, CD34 negative, CD56 positive, TdT negative, and Epstein Barr virus (EBV) positive.

• Hepatosplenic T-cell lymphoma (C) has the following immunophenotype: CD2 positive, CD3 positive, CD4 negative, CD5 negative, CD8 negative/positive, CD19 negative, CD34 negative, CD56 positive/negative, TdT negative, and EBV negative.

• Plasma cell leukemia (D) has the following immunophenotype: CD2 negative, CD3 negative, CD4 negative, CD5 negative, CD8 negative, CD19 negative, CD34 negative, CD56 positive/negative, TdT negative, and EBV negative. In addition, the cells are CD138 positive, CD38 positive (bright), CD79a positive, and CD20 negative/positive.

• Extranodal NK/T-cell lymphoma (E) has the following immunophenotype: CD2 positive, surface CD3 negative, CD4 negative, CD5 negative, CD8 positive/negative, CD19 negative, CD34 negative, CD56 positive, TdT negative, and EBV positive.

Bain BJ, Clark DM, Wilkins BS: Bone Marrow Pathology, 4th ed.West Sussex, UK: Wiley-Blackwell, 2010.

Swerdlow SH, Campo E, Harris NL, et al: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues.Lyon, France: IARC, 2008.

 
* = Required 
* Note Title
* Note