Table 2.
Clinical, survival and treatment data of EBV + TNK-cell lymphoproliferative disease
No | Ohshima category* | Histo | Sex | Ethnicity | Age (yrs) | Tissue type | GEP | HLH | Treatment | Follow up | OS (mth) |
---|---|---|---|---|---|---|---|---|---|---|---|
1 | A1 | P | M | Indonesian | 3.4 | BM | No | + | HLH -P | Alive | 55 |
2 | A1 | P | M | Chinese | 13.1 | BM | No | + | HLH-P, AHCT | Dead (AHCT complications) | 48 |
3 | A1 | P | M | Vietnamese | 24.8 | BM | No | + | Chemo | Alive | 6 |
4 | A2 | P | F | Chinese | 5.3 | BM | No | + | HLH-P, AHCT | Alive | 64 |
5 | A2 | P | M | Chinese | 1.4 | Skin | Yes | + | HLH-P , AHCT | Alive (graft rejection) | 95 |
6 | A2 | P | F | Indonesian | 6.1 | BM | No | + | HLH-P | Alive | 31 |
7 | A2 | P | M | Chinese | 16.7 | LN | Yes | - | Steroids, AHCT | Dead | 14 |
8 | A1 or A2 | P | M | Japanese | 4 | liver | No | + | AHCT | Alive | 94.4 |
9 | A1 or A2 | P | F | Japanese | 19 | BM | No | - | AHCT | Alive | 9.2 |
10 | A1 or A2 | P | F | Japanese | 19 | BM | No | + | Chemo | Alive | 32.5 |
11 | A1 or A2 | P | F | Japanese | 39 | Skin | No | - | Chemo | Dead | 21.8 |
12 | A3 | M | F | Vietnamese | 16.4 | BM | No | + | HLH-P | Dead | 0.4 |
13 | A3 | M | F | Chinese | 4.8 | BM | No | + | HLH-P | Alive | 21 |
14 | B | M | M | Indonesian | 13.3 | BM | No | + | HLH -P | Dead | 0.83 |
15 | B | M | M | Indonesian | 20.9 | BM | No | + | HLH -P | Dead | 2 |
16 | B | M | F | Chinese | 9.5 | LN | Yes | + | Supportive | Dead | 1 |
17 | B | M | M | Chinese | 18.7 | LN | Yes | + | HLH -P | Dead | 1.16 |
18 | B | M | M | Vietnamese | 1 | BM | No | + | HLH-P | NA | NA |
19 | B | M | M | Filipino | 7.6 | BM | No | + | HLH-P | Dead | 1 |
20 | B | M | F | Chinese | 19.3 | BM | No | + | HLH-P | Dead | 0.1 |
21 | B | M | F | Japanese | 48 | BM | No | + | Chemo, AHCT | Dead | 2.2 |
22 | A3 or B | M | M | Others | 18.3 | BM | No | NA | Supportive | Dead | 0.7 |
Abbreviations: M male, F female, Histo histology, P polymorphic, M monomorphic, HLH-P 2004 Histiocytic Lymphohistiocytosis protocol, AHCT Allogeneic hematopoietic cell transplant, + present, − absent, TCR Clonality for TCRG gene, R rearranged, NR not rearranged, NA not available, OS overall survival, LN lymph node, BM bone marrow, Chemo chemotherapy. *Categorization of EBV-associated T/NK LPD in children proposed by Ohshima et al. [13].