Version May 2024
| Entity | Histology | Immunophenotype | Genetic features |
| Angioimmunoblastic T cell lymphoma | Polymorphous infiltrate of small to large atypical lymphocytes with prominent arborizing high endothelial venules. Expanded populations of follicular dendritic cells can be detected with CD21 staining. | Express CD3 and CD4; sometimes express BCL-6 and CD10. May lose a subset of pan-T cell antigens (ie, CD2, CD3, CD5, CD7). | TCR genes are rearranged in 75 to 90%; immunoglobulin heavy chains are rearranged in 25%. Epstein-Barr virus and human herpes virus-6 genomes may be present in reactive B cells. |
| Enteropathy-associated T cell lymphoma* | Tumor cells are of variable size and morphology. The adjacent "normal" mucosa often demonstrates signs of celiac disease. | Typically express CD3 and do not express CD4; most cases also CD8 negative. Large cells within the tumor infiltrate are frequently CD30 positive. | TCR beta gene is clonally rearranged. Many patients have an HLA haplotype associated with celiac disease. 50 to 60% have complex segmental amplification of the 9q34 region. |
| Extranodal NK/T cell lymphoma; nasal type | Polymorphous lymphoid infiltrate of variable morphology that invades the vascular walls, often producing extensive fibrinoid necrosis. | Express CD2, cytoplasmic CD3, CD56, and cytotoxic granule proteins, and are negative for surface CD3. | Clonal Epstein-Barr virus genomes are virtually always present. TCR and immunoglobulin genes are usually germline, consistent with a natural killer cell origin. |
| Hepatosplenic T cell lymphoma* | Malignant cells accumulate within the sinusoids of the liver, spleen, or bone marrow. | Express CD2, surface CD3, CD7, and CD16, and do not express CD4, CD5, or CD8. Express gamma/delta TCR or alpha/beta TCR. | Cytogenetics demonstrating isochrome 7q supports the diagnosis, but is not essential. |
| Subcutaneous panniculitis-like T cell lymphoma* | Subcutaneous infiltrate of atypical lymphocytes involving the fat lobules, but typically sparing the septae, overlying dermis, and epidermis. | Express CD3, CD8, and the alpha/beta TCR, but are negative for CD4, CD56, and TCR gamma. Strong expression of the cytotoxic granule proteins. Expression of CD2, CD5, and/or CD7 is variable. | Clonal TCR rearrangements are present. |
| Anaplastic large cell lymphoma T/null cell type | Composed of large cells with horseshoe-shaped or embryoid nuclei, prominent nucleoli, and abundant cytoplasm. Often shows a cohesive growth pattern. | Homogeneous and strong expression of CD30 in a membrane and golgi pattern. Usually express one or more T cell antigens (ie, CD2, CD3, CD5, CD7). | Two distinct subtypes: ALK positive (detected by immunostains or molecular genetics) and ALK negative. |
| Peripheral T cell lymphoma; not otherwise specified | Mixture of small and large atypical cells. | Varies greatly from case to case, but always demonstrates expression of one or more of the pan-T antigens (ie, CD2, CD3, CD5, CD7). Roughly half of cases lose a T cell antigen (ie, CD2, CD3, CD5, CD7). | While the majority have cytogenetic abnormalities, none is specific. |
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