Table 1.
Clinical and pathological characteristics of included patients.
Case number | Sex/Age at diagnosis | Ann Arbor Stage | Pattern | T-cell phenotype or TFH phenotype | Epstein-Barr virus-encoded RNA (EBER) | Management | Follow-up | Status at last follow-up |
---|---|---|---|---|---|---|---|---|
Case 1 | Male/ 64 | I | AITL patterns 1 & 2 | CD4 + , PD1 + , ICOS+ | Negative | Surgical excision of single site of involvement and observation | 5 years | Alive |
Case 2 | Male/ 69 | II | AITL pattern 1 | CD4 + , PD1 + , ICOS+ | Negative | Observation | 5 years | Alive |
Case 3 | Female/ 70 | I | AITL pattern 1 | CD4 + , CD10 + , PD1 + , ICOS+ | Scattered positive | Excisional LN biopsy of single site of disease | 5 years | Alive |
Case 4 | Female / 72 | I | AITL pattern 1 | PD1 + , ICOS+ | EBER+ in large B cell immunoblasts | Observation | 3 years | Alive |
Case 5 | Female / 67 | III | AITL pattern 1 | CD4+ PD1 + ICOS + | Positive | Observation | 6 years | Alive |
Case 6 | Female / 42 | III | AITL pattern 1 | CD4+ PD1 + ICOS + | Positive | Observation | 5 years | Alive |
Case 7 | Female / 52 | II | AITL patterns 1 & 2 | ICOS+ PD1+ | Positive | Prednisolone and paracetamol | 5 years | Alive |
Case 8 | Female / 74 | IV | AITL pattern 1 | CD4 + CD5-a | Positive | Prednisolone for concomitant diagnosis of ILD | 6 years | Deceased |
Case 9 | Female / 78 | II | AITL pattern 3 | CD4 CD10+ BCL6+ PD1+ | Positive | Prednisolone | 22 months | Deceased |
aCD4 + T-cells with CD5 loss are present in peripheral areas of germinal centers in keeping with a neoplastic Tfh population. Prominent high endothelial venules and presence of focal areas of follicular dendritic cell expansion are seen.