Clinical subdivision of noncutaneous, mature T/NK neoplasms, unique features, and expected 5-year survival.
| . | Unique Features . | Survival (%) . |
|---|---|---|
| *Survival pertains to the acute leukemia and lymphoma presentations of ATLL | ||
| Nodal | ||
| Anaplastic large cell, ALK-positive | t(2;5)(p23;q35) and variants; extranodal involvement (50–80%), skin (21–35%) | 60–90 |
| Anaplastic large cell, ALK-negative | Distinguish from primary cutaneous anaplastic large cell lymphoma (ALCL) | 10–45 |
| Angioimmunoblastic | Autoimmunity | 10–30 |
| Peripheral T-cell lymphoma, unspecified | Most common, survival dependent on IPI | 15–35 |
| Extranodal | ||
| Nasal | Epstein-Barr virus association, central nervous system risk | |
| Localized | 50–70 | |
| Disseminated (nasal type) | Sites: skin, gastrointestinal tract, testis, orbit | 5–10 |
| Enteropathy associated | Celiac disease; small bowel obstruction | 5–20 |
| Hepatosplenic, γδ | Isochromosome 7, trisomy 8; can occur in organ transplants | 5–15 |
| Subcutaneous panniculitis-like | Aggressive with hemophagocytosis; may be indolent | 10–30 |
| Leukemia | ||
| T-Prolymphocytic leukemia | Chromosome 14 abnormalities | 10–20 |
| Adult T-cell lymphoma/leukemia | HTLV-1 association, hypercalcemia. Four types: acute (55–65%) chronic, smoldering leukemia and lymphoma (20–25%) | 0–15* |
| Large granular lymphocytic leukemia | Rheumatoid arthritis, neutropenia | 50–75 |
| Aggressive NK leukemia | May represent leukemic phase of extranodal NK neoplasms (nasal type) | 0–10 |
| . | Unique Features . | Survival (%) . |
|---|---|---|
| *Survival pertains to the acute leukemia and lymphoma presentations of ATLL | ||
| Nodal | ||
| Anaplastic large cell, ALK-positive | t(2;5)(p23;q35) and variants; extranodal involvement (50–80%), skin (21–35%) | 60–90 |
| Anaplastic large cell, ALK-negative | Distinguish from primary cutaneous anaplastic large cell lymphoma (ALCL) | 10–45 |
| Angioimmunoblastic | Autoimmunity | 10–30 |
| Peripheral T-cell lymphoma, unspecified | Most common, survival dependent on IPI | 15–35 |
| Extranodal | ||
| Nasal | Epstein-Barr virus association, central nervous system risk | |
| Localized | 50–70 | |
| Disseminated (nasal type) | Sites: skin, gastrointestinal tract, testis, orbit | 5–10 |
| Enteropathy associated | Celiac disease; small bowel obstruction | 5–20 |
| Hepatosplenic, γδ | Isochromosome 7, trisomy 8; can occur in organ transplants | 5–15 |
| Subcutaneous panniculitis-like | Aggressive with hemophagocytosis; may be indolent | 10–30 |
| Leukemia | ||
| T-Prolymphocytic leukemia | Chromosome 14 abnormalities | 10–20 |
| Adult T-cell lymphoma/leukemia | HTLV-1 association, hypercalcemia. Four types: acute (55–65%) chronic, smoldering leukemia and lymphoma (20–25%) | 0–15* |
| Large granular lymphocytic leukemia | Rheumatoid arthritis, neutropenia | 50–75 |
| Aggressive NK leukemia | May represent leukemic phase of extranodal NK neoplasms (nasal type) | 0–10 |