Enteropathy-Type T Cell Lymphoma

Supplemental Studies

T lineage markers
- CD3 90%
- CD8 30%
  - 80% of CD56+ cases express CD8
- CD4, CD5 negative
Cytotoxic protein markers
- TIA1, perforin, Granzyme B positive in nearly all cases
CD103 positive in most cases
- Lymphoid adhesion molecule of enteric T cell subset
CD56 20% positive
- More often in small to medium sized cases
CD30 40%
- All in CD56+ subset
EMA 16%
- All in CD56+ subset
CD79a reported in 3/52 cases
- All expressed cytotoxic markers
- All were CD20 negative
Negative:
- CD20
- CD57
- ALK

EBV EBER variably reported
- 0% in US patients (Ilas et al.)
- 7% in German patients (Chott et al.)
- 100% in Mexican patients (Quintanilla-Martinez et al.)
T cell receptor beta and gamma genes clonally rearranged
- Same clonal rearrangements found in adjacent mucosa
- Cases of refractory sprue may show clonal rearrangements, suggesting a precursor lesion
Various chromosomal gains and losses
- Most common is gain of 9q33-34 in 64% of cases
  - Very rare in other lymphomas