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18 Cards in this Set

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Leukemic/disseminated mature T-cell/NK-cell neoplasms include (4)
- T-PLL
- T-LGL (large granular lymphocytic leukemia)
- Aggressive NK cell leukemia
- Adult T-cell leukemia/lymphoma
Cutaneous mature T/NK-cell neoplasm
- MF (mycosis fungoides)
- Sezary syndrome
- primary cutaneous ALCL
- lymphomatoid papulosis
Other extranodal mature T/NK-cell neoplasms (lymphomas)
- extranodal NK/T cell lymphoma, nasal type
- enteropathy-type T-cell lymphoma
- hepatosplenic T-cell lymphoma
- subcutaneous panniculitis-like T-cell lymphoma
Nodal mature T/NK-cell neoplasms (lymphomas)
- angioimmunoblastic T-cell lymphoma
- peripheral T-cell lymphoma, unspecified
- ALCL
Blastic NK cell lymphoma is a neoplasm of __ lineage and stage of differentiation
uncertain!!
mature T-cell neoplasms are derived from __ or __ T cells
- mature or post-thymic
Mature NK/T-cell neoplasms are relatively uncommon, but of these, ____ and ___ are the most common subtypes
- peripherl T-cell lymphoma, unspecified
- ALCL
In general, T-cell lymphomas are more common in __ populations
Asian
Two main types of T cells (based on TCR type)
- alpha-beta
- gamma-delta

- both associate with CD3
CD3 contains __, __, and __ subunits, of which, NK-cells only possess __
- gamma, delta, and epsilon subunits
- NK-cells only express the epsilon subunit in the CYTOPLASM only
-- this NK-cell cytoplasmic CD3 epsilon subunit can be detected by polyclonal antibodies to CD3

NK-cells lack surface CD3!!
Gamma-delta T-cells represent a more primitive type of ___. They are restricted to ___ sites. Role they play:
- immune response
- epithelial sites (splenic red pulp, intestinal epithelium, other epithelial sites) (sites that are more often affected by gamma-delta T-cell lymphomas)
- they have restricted antigen recognition, but are NOT MHC restricted in their function
- first line of defense against bacterial peptides (heat shock proteins); mycobacterial infections, mucosal immunity
Gamma-delta T-cells are:
CD4__
CD8__
CD5__
- CD4- and CD8-
- usually CD5- too!

- subpopulation is CD8+
Gamma-delta T-cells comprise __% of the normal T cells
<5%
Many clinical manifestations related to NK/T-cell neoplasms are due to tumor cytokine expression
- ATLL: hypercalcemia due to osteoclast-activating factors
- NK/T-cell neoplasms in general: hemophagocytic syndrome
NK cells share some functions and markers with ___ T cells
- cytoxic T cells

CD2+
CD7+
CD8+
Markers less often seen in T-cells:
CD56+
CD57+
CD16+

- BUT!!! sCD3-
- often cCD3epsilon+
There are __ specific immunophenotypic profiles for most T-cell lymphoma subtypes
NOT!!
In contrast to B-cell neoplasms, in T-cell neoplasm there is __ immunophenotypic evidence of monoclonality
- NOT!
- However, aberrant marker expression may suggest monoclonality
- molecular techniques (PCR) are required - evaluate TCR gene rearrangement
In general, the prognosis for NK/T-cell neoplasms is __
- poor
- aggressive disease with poorer response to therapy
- one exception: ALCL has a better response to therapy
- few clinical trials (relative rarity)
- many treatments are B-cell therapies empirically applied to T-cell diseases