Table 1.

Clinicopathological characteristics of SPTCLs according to HAVCR2 genotype

CharacteristicWTY82CUnknownTotalP
Clinical features 
 Age      
  Median (range), y 40 (16-74) 26 (8-62) 52 (35-68) 32 (8-74) .002* 
  <30 y 6 (25.0%) 18 (72.0%) 0 (0.0%) 24 (45.3%) .001 
  ≥30 y 18 (75.0%) 7 (28.0%) 4 (100.0%) 29 (54.7%)  
 Sex     .404 
  Male 6 (25.0%) 9 (36.0%) 1 (25.0%) 16 (30.2%)  
  Female 18 (75.0%) 16 (64.0%) 3 (75.0%) 37 (69.8%)  
 HLH/HLH–like systemic illness     <.001 
  No 22 (95.7%) 11 (45.8%) 2 (100.0%) 35 (71.4%)  
  Yes 1 (4.3%) 13 (54.2%) 0 (0.0%) 14 (28.6%)  
 BM involvement     1.000 
  Absent 21 (100.0%) 12 (95.7%) 0 (0.0%) 43 (93.5%)  
  Present 0 (0.0%) 1 (4.3%) 2 (100.0%) 3 (6.5%)  
 First-line treatments     .069 
  Chemotherapy 16 (88.9%) 14 (63.6%) 3 (100.0%) 33 (76.7%)  
  Immunosuppressive 2 (11.1%) 8 (36.4%) 0 (0.0%) 10 (23.3%)  
 HSCT§     .070 
  No 8 (80.0%) 3 (33.3%) 2 (100.0%) 13 (61.9%)  
  Yes 2 (20.0%) 6 (66.7%) 0 (0.0%) 8 (38.1%)  
 Relapse     .345 
  No 17 (73.9%) 14 (60.9%) 3 (100.0%) 34 (69.4%)  
  Yes 6 (26.1%) 9 (39.1%) 0 (0.0%) 15 (30.6%)  
 5-y RFS     .001* 
  Median (range), m 57 (1-60) 11 (1-60) 60 (16-60) 30 (1-60)  
 Death     1.000 
  No 21 (87.5%) 21 (87.5%) 3 (100.0%) 45 (88.2%)  
  Yes 3 (12.5%) 3 (12.5%) 0 (0.0%) 6 (11.8%)  
 5-y OS     .004* 
  Median (range), m 60 (2-60) 25 (1-60) 60 (16-60) 39 (1-60)  
 5-y DSS     .003* 
  Median (range), m 59.5 (2-60) 19 (1-60) 60 (16-60) 37 (1-60)  
Histopathological features 
 Necrosis     .314 
  Absent 14 (66.7%) 13 (52.0%) 1 (50.0%) 28 (58.3%)  
  Present 7 (33.3%) 12 (48.0%) 1 (50.0%) 20 (41.7%)  
 Granulomatous inflammation     .037 
  Absent 17 (81.0%) 25 (100.0%) 2 (100.0%) 44 (91.7%)  
  Present 4 (19.0%) 0 (0.0%) 0 (0.0%) 4 (8.3%)  
 Lipogranuloma     .293 
  Absent 16 (76.2%) 22 (88.0%) 2 (100.0%) 40 (83.3%)  
  Present 5 (23.8%) 3 (12.0%) 0 (0.0%) 8 (16.7%)  
  pSTAT3 IHC     .015 
  Low 12 (70.6%) 4 (25.0%) 0 (0.0%) 16 (48.5%)  
  High 5 (29.4%) 12 (75.0%) 0 (0.0%) 17 (51.5%)  
 CCR4 IHC     .019 
  Low 5 (27.8%) 12 (66.7%) 0 (0.0%) 17 (47.2%)  
  High 13 (72.2%) 6 (33.3%) 0 (0.0%) 19 (52.8%)  
Total  24 (45.3%) 25 (47.2%) 4 (7.5%) 53 (100.0%)  
CharacteristicWTY82CUnknownTotalP
Clinical features 
 Age      
  Median (range), y 40 (16-74) 26 (8-62) 52 (35-68) 32 (8-74) .002* 
  <30 y 6 (25.0%) 18 (72.0%) 0 (0.0%) 24 (45.3%) .001 
  ≥30 y 18 (75.0%) 7 (28.0%) 4 (100.0%) 29 (54.7%)  
 Sex     .404 
  Male 6 (25.0%) 9 (36.0%) 1 (25.0%) 16 (30.2%)  
  Female 18 (75.0%) 16 (64.0%) 3 (75.0%) 37 (69.8%)  
 HLH/HLH–like systemic illness     <.001 
  No 22 (95.7%) 11 (45.8%) 2 (100.0%) 35 (71.4%)  
  Yes 1 (4.3%) 13 (54.2%) 0 (0.0%) 14 (28.6%)  
 BM involvement     1.000 
  Absent 21 (100.0%) 12 (95.7%) 0 (0.0%) 43 (93.5%)  
  Present 0 (0.0%) 1 (4.3%) 2 (100.0%) 3 (6.5%)  
 First-line treatments     .069 
  Chemotherapy 16 (88.9%) 14 (63.6%) 3 (100.0%) 33 (76.7%)  
  Immunosuppressive 2 (11.1%) 8 (36.4%) 0 (0.0%) 10 (23.3%)  
 HSCT§     .070 
  No 8 (80.0%) 3 (33.3%) 2 (100.0%) 13 (61.9%)  
  Yes 2 (20.0%) 6 (66.7%) 0 (0.0%) 8 (38.1%)  
 Relapse     .345 
  No 17 (73.9%) 14 (60.9%) 3 (100.0%) 34 (69.4%)  
  Yes 6 (26.1%) 9 (39.1%) 0 (0.0%) 15 (30.6%)  
 5-y RFS     .001* 
  Median (range), m 57 (1-60) 11 (1-60) 60 (16-60) 30 (1-60)  
 Death     1.000 
  No 21 (87.5%) 21 (87.5%) 3 (100.0%) 45 (88.2%)  
  Yes 3 (12.5%) 3 (12.5%) 0 (0.0%) 6 (11.8%)  
 5-y OS     .004* 
  Median (range), m 60 (2-60) 25 (1-60) 60 (16-60) 39 (1-60)  
 5-y DSS     .003* 
  Median (range), m 59.5 (2-60) 19 (1-60) 60 (16-60) 37 (1-60)  
Histopathological features 
 Necrosis     .314 
  Absent 14 (66.7%) 13 (52.0%) 1 (50.0%) 28 (58.3%)  
  Present 7 (33.3%) 12 (48.0%) 1 (50.0%) 20 (41.7%)  
 Granulomatous inflammation     .037 
  Absent 17 (81.0%) 25 (100.0%) 2 (100.0%) 44 (91.7%)  
  Present 4 (19.0%) 0 (0.0%) 0 (0.0%) 4 (8.3%)  
 Lipogranuloma     .293 
  Absent 16 (76.2%) 22 (88.0%) 2 (100.0%) 40 (83.3%)  
  Present 5 (23.8%) 3 (12.0%) 0 (0.0%) 8 (16.7%)  
  pSTAT3 IHC     .015 
  Low 12 (70.6%) 4 (25.0%) 0 (0.0%) 16 (48.5%)  
  High 5 (29.4%) 12 (75.0%) 0 (0.0%) 17 (51.5%)  
 CCR4 IHC     .019 
  Low 5 (27.8%) 12 (66.7%) 0 (0.0%) 17 (47.2%)  
  High 13 (72.2%) 6 (33.3%) 0 (0.0%) 19 (52.8%)  
Total  24 (45.3%) 25 (47.2%) 4 (7.5%) 53 (100.0%)  

BM, bone marrow; DSS, disease-specific survival; HLH, hemophagocytic lymphohistiocytosis; HSCT, hematopoietic stem cell transplantation; OS, overall survival; RFS, relapse-free survival.

*

Compared between HAVCR2WT and HAVCR2Y82C by using the Mann-Whitney U test.

Compared between HAVCR2WT and HAVCR2Y82C by using the χ2 test.

Compared between HAVCR2WT and HAVCR2Y82C by using Fisher’s exact test.

§

Either autologous or allogeneic transplantation.

Used median values as cutoffs.

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