Proliferations of large granular lymphocytes are a heterogeneous group of conditions composed of either T lymphocytes or natural killer (NK) cells. These may be reactive or clonal proliferations. McDaniel et al proposed a classification based on immunophenotype and clinical findings.
Shared characteristics:
(1) proliferation of lymphocytes with a relative abundance of cytoplasm that contains several azurophilic granules
(2) CD2 positive
|
|
CD3 |
CD16 |
CD57 |
|
T cell, Type 1 |
positive |
negative |
positive |
|
T cell, Type 2 |
positive |
positive |
positive |
|
NK cell, Type 1 |
negative |
positive |
positive |
|
NK cell, Type 2 |
negative |
positive |
negative |
where:
• CD16 distinguishes T cell types.
• CD57 distinguishes NK cell types.
|
Features |
T Cell, Type 1 |
T Cell, Type 2 |
|
clinically aggressive |
no |
no |
|
associated with rheumatoid arthritis |
may be |
may be |
|
associated with autoimmunity |
may be |
may be |
|
neutropenia |
may be present |
may be present |
|
recurrent infections |
may occur |
may occur |
|
organomegaly |
may be present |
may be present |
|
T cell receptor (beta chain gene) |
clonal or germline |
clonal |
|
NK cell activity |
absent |
may be present |
|
antibody-dependent cellular cytotoxicity |
absent |
present |
|
Features |
NK Cell, Type 1 |
NK Cell, Type 2 |
|
clinically aggressive |
no |
possibly |
|
associated with rheumatoid arthritis |
no |
no |
|
associated with autoimmunity |
no |
no |
|
neutropenia |
usually absent |
usually absent |
|
recurrent infections |
no |
no |
|
organomegaly |
may be present |
may be present |
|
T cell receptor (beta chain gene) |
germline |
germline |
|
NK cell activity |
present |
present |
|
antibody-dependent cellular cytotoxicity |
present |
present |
Specialty: Hematology Oncology
