Cytogenetics of PTCL subtypes
| . | TCR . | Chromosomal/histopathologic features . | Distinguishing features . | 5-year survival, % . |
|---|---|---|---|---|
| PTCL-NOS | αβ | Loss 13q22.3 adverse prognosis; gains 8q,9p,19q,loss 3q,9p | Heterogeneous, variable morphology | 20-30 |
| AITL | αβ | Follicular dendritic cell signature CXCL13+, PD-1+; gains 2,5,13q22.3 adverse prognosis | Immunodeficiency and immune dysregulation Helper B cells may respond to cyclosporine | 32 |
| ALCL ALK+ | αβ | (2,5) translocation; gain 1q, loss6q, 13q | Bone marrow involvement in only 29%, median age 34 y | 70 |
| ALK ALK− | αβ | Pax 5−, CD15− | Median age 58 y | 49 |
| NK-/T-cell | CD56+, often EBV+ | Stage I/II patients respond to radiotherapy or radiochemotherapy Serum EBV copy number predictive of outcome | Nasal type 64 Extranasal type < 20 | |
| SPTCL- αβ SPTCL- γδ | Αβ γδ | CD3+CD8+CD56−5q,13q gains CD3+CD8− CD56+/− | Nodules and plaques nodules, often ulceration Hemophagocytic syndrome | 82; 11 |
| Hepatosplenic T-cell lymphoma | γδ | Isochromosme 7q, CD3+, CD4−CD8−, may be CD56+ | Infiltration of sinusoids in liver, spleen, bone marrow Erythrophagocytosis | 2-year OS 20 |
| EATL | αβ | Gains at chromosome 9q33-q34 CD3+ CD7+, may be CD8+ CD56+ with monomorphic type | Associated with celiac sprue, malabsorption | 4 |
| . | TCR . | Chromosomal/histopathologic features . | Distinguishing features . | 5-year survival, % . |
|---|---|---|---|---|
| PTCL-NOS | αβ | Loss 13q22.3 adverse prognosis; gains 8q,9p,19q,loss 3q,9p | Heterogeneous, variable morphology | 20-30 |
| AITL | αβ | Follicular dendritic cell signature CXCL13+, PD-1+; gains 2,5,13q22.3 adverse prognosis | Immunodeficiency and immune dysregulation Helper B cells may respond to cyclosporine | 32 |
| ALCL ALK+ | αβ | (2,5) translocation; gain 1q, loss6q, 13q | Bone marrow involvement in only 29%, median age 34 y | 70 |
| ALK ALK− | αβ | Pax 5−, CD15− | Median age 58 y | 49 |
| NK-/T-cell | CD56+, often EBV+ | Stage I/II patients respond to radiotherapy or radiochemotherapy Serum EBV copy number predictive of outcome | Nasal type 64 Extranasal type < 20 | |
| SPTCL- αβ SPTCL- γδ | Αβ γδ | CD3+CD8+CD56−5q,13q gains CD3+CD8− CD56+/− | Nodules and plaques nodules, often ulceration Hemophagocytic syndrome | 82; 11 |
| Hepatosplenic T-cell lymphoma | γδ | Isochromosme 7q, CD3+, CD4−CD8−, may be CD56+ | Infiltration of sinusoids in liver, spleen, bone marrow Erythrophagocytosis | 2-year OS 20 |
| EATL | αβ | Gains at chromosome 9q33-q34 CD3+ CD7+, may be CD8+ CD56+ with monomorphic type | Associated with celiac sprue, malabsorption | 4 |
PTCL indicates peripheral T-cell lymphoma; TCR, T-cell receptor; NOS, not otherwise specified; AITL, angioimmunoblastic T-cell lymphoma; ALCL, anaplastic large cell lymphoma; NK, natural killer; SPTCL, subcutaneous panniculitis-like T-cell lymphoma; and EATL, enteropathy-associated T-cell lymphoma.