Chronic Lymphoproliferative Disorders of NK Cells

This is considered a provisional entity by the WHO classification in 2008.   In many ways this entity is similar clinically to T-LGL leukemia, but there are no rearrangements of immunoglobulin or T-cell receptors since the cells are NK cells.
Characteristic Immunophenotypic Profile
Marker
Comment
Surface CD3 negative.  Cytoplasmic CD3ε is often positive.
Usually weak expression
TIA-1
Positive
Positive
+/- May show diminished of loss of expression.
+/- May show diminished of loss of expression.
CD57
+/- May show diminished of loss of expression.
May show aberrant uniform expression.
References
Hematopathology. [edited by] Jaffe, ES. 1st. ed. Elsevier, Inc. © 2011.