Clinical and pathologic features used to distinguish primary cutaneous anaplastic lymphoma and systemic anaplastic large cell lymphoma (ALCL), ALK-positive and ALK-negative subtypes
| Features . | ALK- Positive Systemic ALCL . | ALK- Negative Systemic ALCL . | Primary Cutaneous ALCL . |
|---|---|---|---|
| Cytotoxic proteins: granzyme B, perforin, TIA-1 (T-cell intracytoplasmic antigen). | |||
| * Occasional cases ALCL CD8+/CD4− | |||
| Abbreviations: ALK, anaplastic lymphoma kinase; EMA, epithelial membrane antigen; OS, overall survival | |||
| T-cell phenotype* | CD4+ | CD4+ | CD4+ |
| ALK protein | + | − | − |
| CD30 | + | + | + |
| Clusterin | + | + | − |
| EMA | + | −/+ | − |
| Cytotoxic proteins | + (80%) | + (50%) | + (70%) |
| Median age | < 30 | > 50 | > 50 |
| Sex | M > F | M = F | M > F |
| 5-year OS | 65%–90% | 30%–40% | > 90% |
| Features . | ALK- Positive Systemic ALCL . | ALK- Negative Systemic ALCL . | Primary Cutaneous ALCL . |
|---|---|---|---|
| Cytotoxic proteins: granzyme B, perforin, TIA-1 (T-cell intracytoplasmic antigen). | |||
| * Occasional cases ALCL CD8+/CD4− | |||
| Abbreviations: ALK, anaplastic lymphoma kinase; EMA, epithelial membrane antigen; OS, overall survival | |||
| T-cell phenotype* | CD4+ | CD4+ | CD4+ |
| ALK protein | + | − | − |
| CD30 | + | + | + |
| Clusterin | + | + | − |
| EMA | + | −/+ | − |
| Cytotoxic proteins | + (80%) | + (50%) | + (70%) |
| Median age | < 30 | > 50 | > 50 |
| Sex | M > F | M = F | M > F |
| 5-year OS | 65%–90% | 30%–40% | > 90% |