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peripheral T-cell lymphoid neoplasm
Category includes heterogeneous diseases.
Classification:
1) working formulation:
- diffuse small cleaved cell lymphoma
- large cell immunoblastic lymphoma
- diffuse mixed small & large cell lymphoma
2) Kiel classification:
- T-zone lymphoma,
- lymphoepithelioid cell lymphoma
Microscopic pathology:
Immunophenotype: T-cell associated antigens variable CD2 +/-, CD3 +/-, CD5 +/- CD7 -/+, CD4 > CD8.
Genetics: TCR usually rearranged, Ig usually germline
Management:
- treated with combination chemotherapy [1]
Interactions
disease interactions
Specific
adult T-cell lymphoma/leukemia (ATL/L)
anaplastic large cell lymphoma (ALCL), CD30+, T-cell type
angiocentric T-cell lymphoma (lymphomatoid granulomatosis, polymorphic reticulosis, midline malignant reticulocytosis, Stewart's granuloma)
angioimmunoblastic T-cell lymphoma (AILD)
cutaneous T-cell lymphoma (CTCL)
intestinal T-cell lymphoma
T-cell prolymphocytic leukemia (TPLL)
unspecified primary cutaneous peripheral T-cell lymphoma
General
T-cell lymphoid neoplasm (T-cell leukemia, T-cell lymphoma)
References
- Medical Knowledge Self Assessment Program (MKSAP) 18,
American College of Physicians, Philadelphia 2018