Table 1.

Subtype, OS, and mutational profile of North American ATLL

ATL no.SexAge at diagnosis, ySubtypeOS, dMutationsMutations, nTreatment
1 Male 83 A 33 KIT, APC, MYC, TP53, DDX3X, TSC1 5 Unknown 
2 Male 59 L 382 NRAS, POT1, CEBPA, KEAP1, AR, NOTCH1, MCL1 5 Etoposide, cyclophosphamide, cytarabine, cisplatin 
3 Female 28 A 543 ALK, BCL6, RIPK1, GATA3, IGF1R, TP53, CEBPA 7 CHOP, cytarabine, denileukin diftitox, IT MTX 
4 Female 66 L 363 NOTCH2, DDR2, GATA3 3 EPOCH 
5 Female 74 L 160 KDR, FAT1, FGFR2, AKT1, TP53, ERBB2, MED12 7 EPOCH 
6 Male 53 A 88 XPO1, TBL1XR1, PDGFRA, KIT, FGFR4, EP300, RICTOR 6 EPOCH (died after cycle 1) 
7 Female 48 L 225 KDR, CARD11, NKX2-1(2) 4 Cyclophosphamide, doxorubicin, etoposide, MTX 
8 Female 64 L 215 TNFAIP3, TSC1, BRCA2, NOTCH1 3 Cyclophosphamide, doxorubicin, etoposide 
9 Female 63 L 739 CARD11, NOTCH1, KRAS, POT1 3 EPOCH 
10 Male 36 L 52 CEBPA, RHOA, PBRM1, CD79A, STAG2 5 Bortezomib, cytarabine, doxorubicin, etoposide 
11 Female 70 L 451 NRAS, VHL, APC, EGFR, PTCH1, ASXL1, KMT2A 6 DA EPOCH bortezomib raltegravir 
12 Female 54 L 23 FAT1, HIST1H1E, PLCG2 3 CHOP died after cycle 1 
13 Female 37 A 17 FGFR4, APC, FAT1, SPEN 3 EPOCH 
14 Male 41 L 771* EZH2, DDX3X, FAT1, PTCH1 4 EPOCH + allogeneic transplant 
15 Male 51 L 312 TP53, EP300, PALB2, PTCH1, TRAF3 5 EPOCH 
16 Female 57 C 2554* APC, PDGFRB, TET2, DNMT3A 3 IFN-AZT 
17 Male 36 A 75 KDR, NOTCH1, TP53, SPOP 3 HyperCVAD, CHOP, EPOCH (1 cycle each) 
18 Female 67 A 175 AKT2, CDKN2A, EP300, FAT1, FBXW7, FLT3, PBRM1, SPEN 7 EPOCH + raltegravir + bortezomib + IFN 
19 Male 65 A 392 SETBP1, EP300 2 EPOCH 
20 Male 74 A 1191* FGFR3, FAT1 2 EPOCH 1 cycle, IFN 
21 Female 37 C/S 6345* CDH1, EP300 2 Treated initially as mycosis fungoides, followed by brentuximab 
22 Male 60 C/S 126 AKT2, ALK, CD79A, EP300, ZMYM3, AR 6 None, due to poor performance status and comorbidities 
23 Female 54 A 503* DDR2, IDH1, TBL1XR1, TP53, ZMYM3 5 ESHAP 1 cycle, followed by EPOCH 
24 Female 59 L 176 FGFR3, FLT1, KRAS, P2RY8, TET2 5 EPOCH + raltegravir + bortezomib, palliative after cycle 
25 Male 64 A 73 AR, JAK3, PDGFRA, SETBP1, TP53, XPO1, ZFHX4† 7 EPOCH + raltegravir + bortezomib, died after cycle 2 
26 Female 61 S 102 AKT1, AXL,†PALB2 2 None 
27 Female 40 L 351* BCL6, MDM4† 2 EPOCH + raltegravir + bortezomib 
28 Male 40 A 322 CDH1, HIST1H1E, NOTCH1 3 EPOCH + raltegravir + bortezomib 
29 Female 63 A 192 NOTCH1, APC, APC, GATA2, KLF2,†NTRK1, SMARCB1, SPEN, TBL1XR1, TCF3 10 IFN-AZT, followed by 2 cycles ESHAP, followed by ICE + bortezomib, followed by a clinical trial 
30 Female 70 A 153* ALK, ALK, CDKN2A, ERBB3, FAS,†FAT1, HRAS, KLF2,†PIK3CD, PIK3CD, TBL1XR1 11 EPOCH 
ATL no.SexAge at diagnosis, ySubtypeOS, dMutationsMutations, nTreatment
1 Male 83 A 33 KIT, APC, MYC, TP53, DDX3X, TSC1 5 Unknown 
2 Male 59 L 382 NRAS, POT1, CEBPA, KEAP1, AR, NOTCH1, MCL1 5 Etoposide, cyclophosphamide, cytarabine, cisplatin 
3 Female 28 A 543 ALK, BCL6, RIPK1, GATA3, IGF1R, TP53, CEBPA 7 CHOP, cytarabine, denileukin diftitox, IT MTX 
4 Female 66 L 363 NOTCH2, DDR2, GATA3 3 EPOCH 
5 Female 74 L 160 KDR, FAT1, FGFR2, AKT1, TP53, ERBB2, MED12 7 EPOCH 
6 Male 53 A 88 XPO1, TBL1XR1, PDGFRA, KIT, FGFR4, EP300, RICTOR 6 EPOCH (died after cycle 1) 
7 Female 48 L 225 KDR, CARD11, NKX2-1(2) 4 Cyclophosphamide, doxorubicin, etoposide, MTX 
8 Female 64 L 215 TNFAIP3, TSC1, BRCA2, NOTCH1 3 Cyclophosphamide, doxorubicin, etoposide 
9 Female 63 L 739 CARD11, NOTCH1, KRAS, POT1 3 EPOCH 
10 Male 36 L 52 CEBPA, RHOA, PBRM1, CD79A, STAG2 5 Bortezomib, cytarabine, doxorubicin, etoposide 
11 Female 70 L 451 NRAS, VHL, APC, EGFR, PTCH1, ASXL1, KMT2A 6 DA EPOCH bortezomib raltegravir 
12 Female 54 L 23 FAT1, HIST1H1E, PLCG2 3 CHOP died after cycle 1 
13 Female 37 A 17 FGFR4, APC, FAT1, SPEN 3 EPOCH 
14 Male 41 L 771* EZH2, DDX3X, FAT1, PTCH1 4 EPOCH + allogeneic transplant 
15 Male 51 L 312 TP53, EP300, PALB2, PTCH1, TRAF3 5 EPOCH 
16 Female 57 C 2554* APC, PDGFRB, TET2, DNMT3A 3 IFN-AZT 
17 Male 36 A 75 KDR, NOTCH1, TP53, SPOP 3 HyperCVAD, CHOP, EPOCH (1 cycle each) 
18 Female 67 A 175 AKT2, CDKN2A, EP300, FAT1, FBXW7, FLT3, PBRM1, SPEN 7 EPOCH + raltegravir + bortezomib + IFN 
19 Male 65 A 392 SETBP1, EP300 2 EPOCH 
20 Male 74 A 1191* FGFR3, FAT1 2 EPOCH 1 cycle, IFN 
21 Female 37 C/S 6345* CDH1, EP300 2 Treated initially as mycosis fungoides, followed by brentuximab 
22 Male 60 C/S 126 AKT2, ALK, CD79A, EP300, ZMYM3, AR 6 None, due to poor performance status and comorbidities 
23 Female 54 A 503* DDR2, IDH1, TBL1XR1, TP53, ZMYM3 5 ESHAP 1 cycle, followed by EPOCH 
24 Female 59 L 176 FGFR3, FLT1, KRAS, P2RY8, TET2 5 EPOCH + raltegravir + bortezomib, palliative after cycle 
25 Male 64 A 73 AR, JAK3, PDGFRA, SETBP1, TP53, XPO1, ZFHX4† 7 EPOCH + raltegravir + bortezomib, died after cycle 2 
26 Female 61 S 102 AKT1, AXL,†PALB2 2 None 
27 Female 40 L 351* BCL6, MDM4† 2 EPOCH + raltegravir + bortezomib 
28 Male 40 A 322 CDH1, HIST1H1E, NOTCH1 3 EPOCH + raltegravir + bortezomib 
29 Female 63 A 192 NOTCH1, APC, APC, GATA2, KLF2,†NTRK1, SMARCB1, SPEN, TBL1XR1, TCF3 10 IFN-AZT, followed by 2 cycles ESHAP, followed by ICE + bortezomib, followed by a clinical trial 
30 Female 70 A 153* ALK, ALK, CDKN2A, ERBB3, FAS,†FAT1, HRAS, KLF2,†PIK3CD, PIK3CD, TBL1XR1 11 EPOCH 

A, acute; AZT, azidothymidine; CHOP, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin hydrochloride), vincristine (oncovin), and prednisone; DA EPOCH, dose-adjusted etoposide, prednisone, vincristine, cyclophopshamide, doxorubicin; EPOCH, etoposide, prednisone, vincristine (oncovin), cyclophosphamide, and doxorubicin hydrochloride (hydroxydaunorubicin hydrochloride); ESHAP, etoposide, methylprednisone, cytarabine, cisplatin; ICE, ifosfamide, carboplatin, etoposide; IFN, interferon; IT, intrathecal; L, lymphomatous; MTX, methotrexate.

*

Alive.

†

Genes that were identified as part of the extended spectrum of 236 genes in patients ATL24 to ATL30.

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