Table 1.

Clinicopathological characteristics of 31 pediatric patients with B-cell mPTLD

PTLD-DLBCL, n = 24PTLD-BL, n = 7Total, n = 31
Mean age (range, years old) 8 (2-17) 12 (6-16) 9 (2-17) 
Male 13/24 (54%) 5/7 (71%) 18/31 (58%) 
Female 11/24 (46%) 2/7 (29%) 13/31 (42%) 
Localization    
Extranodal involvement 17/23 (74%) 6/7 (86%) 23/30 (77%) 
Gastrointestinal tract 17/23 (74%) 4/6 (67%) 21/29 (72%) 
Stage    
Stage I 1/19 (5%) 1/7 (14%) 2/26 (8%) 
Stage II 3/19 (16%) 0/7 (0%) 3/26 (11%) 
Stage III 14/19 (74%) 2/7 (29%) 16/26 (62%) 
Stage IV 1/19 (5%) 4/7 (57%) 5/26 (19%) 
COO (NanoString and/or Hans algorithm)     
GCB 2/22 (9%) 7/7 (100%) 9/29 (31%) 
ABC/non-GCB 20/22 (91%) 0/7 (0%) 20/29 (69%) 
EBER hybridization positive 21/23 (91%) 7/7 (100%) 28/30 (93%) 
EBV replication 6/11 (55%) 0/5 (0%) 6/16 (38%) 
Latency pattern    
Latency I 2/20 (10%) 6/6 (100%) 8/26 (31%) 
Latency II 6/20 (30%) 0/6 (0%) 6/26 (23%) 
Latency III  12/20 (60%) 0/6 (0%) 12/26 (46%) 
Mean time from the transplantation to PTLD diagnosis (range, months) 24 (2-141) 74.4 (32-170) 35 (2-170) 
Early-onset PTLD 15/24 (63%) 0/7 (0%) 15/31 (48%) 
Alive with no evidence of disease 17/24 (71%) 7/7 (100%) 24/31 (77%) 
Died of disease 5/24 (21%) 0/7 (0%) 5/31 (16%) 
Fulminant PTLD 3/5 (60%) 0/0 (0%) 3/5 (60%) 
Follow-up (median) (range) 1.3 y (10 d-14.1 y) 5.2 y (2.1-22.6 y) 2.7 y (10 d-22.6 y) 
5y-DFS 78.9% 100% 83.7% 
PTLD-DLBCL, n = 24PTLD-BL, n = 7Total, n = 31
Mean age (range, years old) 8 (2-17) 12 (6-16) 9 (2-17) 
Male 13/24 (54%) 5/7 (71%) 18/31 (58%) 
Female 11/24 (46%) 2/7 (29%) 13/31 (42%) 
Localization    
Extranodal involvement 17/23 (74%) 6/7 (86%) 23/30 (77%) 
Gastrointestinal tract 17/23 (74%) 4/6 (67%) 21/29 (72%) 
Stage    
Stage I 1/19 (5%) 1/7 (14%) 2/26 (8%) 
Stage II 3/19 (16%) 0/7 (0%) 3/26 (11%) 
Stage III 14/19 (74%) 2/7 (29%) 16/26 (62%) 
Stage IV 1/19 (5%) 4/7 (57%) 5/26 (19%) 
COO (NanoString and/or Hans algorithm)     
GCB 2/22 (9%) 7/7 (100%) 9/29 (31%) 
ABC/non-GCB 20/22 (91%) 0/7 (0%) 20/29 (69%) 
EBER hybridization positive 21/23 (91%) 7/7 (100%) 28/30 (93%) 
EBV replication 6/11 (55%) 0/5 (0%) 6/16 (38%) 
Latency pattern    
Latency I 2/20 (10%) 6/6 (100%) 8/26 (31%) 
Latency II 6/20 (30%) 0/6 (0%) 6/26 (23%) 
Latency III  12/20 (60%) 0/6 (0%) 12/26 (46%) 
Mean time from the transplantation to PTLD diagnosis (range, months) 24 (2-141) 74.4 (32-170) 35 (2-170) 
Early-onset PTLD 15/24 (63%) 0/7 (0%) 15/31 (48%) 
Alive with no evidence of disease 17/24 (71%) 7/7 (100%) 24/31 (77%) 
Died of disease 5/24 (21%) 0/7 (0%) 5/31 (16%) 
Fulminant PTLD 3/5 (60%) 0/0 (0%) 3/5 (60%) 
Follow-up (median) (range) 1.3 y (10 d-14.1 y) 5.2 y (2.1-22.6 y) 2.7 y (10 d-22.6 y) 
5y-DFS 78.9% 100% 83.7% 

ABC, activated B cell; EBER, Epstein-Barr virus-encoded small RNAs.

Unclassified cases by NanoString analysis were classified according to Hans algorithm results.

In the absence of LMP-1 assessability, 2 cases with latency pattern IIb-III are included in latency pattern III group.

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