Enteropathy type T cell Lymphoma (EATL)
Epidemiology and Etiology:
· Associated with Celiac
Common sites:
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Gross features:
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Histologic features:
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Immunophenotype:
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Marker: |
Sensitivity: |
Specificity: |
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CD2 |
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CD3 |
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CD5 (neg) |
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CD7 |
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CD4 (neg) |
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CD8 |
+/- |
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CD30 |
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CD103 (HML-1) |
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TdT (neg) |
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Molecular features:
· TCR genes rearranged
· TRB@ and TRG@
· HLADQA1*0501,DQB1*0201 genotype (germline)
associated with Celiac and EATL
· 9q31.3-qter amplification or 16q12.1 deletion (58-70%)
· 1q gain (classical variant)
· 5q gain (classical variant)
· 8q24 amplification (MYC) (monomorphic variant)
· 16q12.1 deletion
Other features:
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References:
· WHO Blue book (2008)