Table 1.

Frequently mutated genes in TCR signaling and epigenetic pathways in T-cell lymphomas

Gene, mutationChange in activityFrequency, %
AITL   
 RHOA, G17V LOF12,31 /unknown29  42-6712,23,28,52,82  
 CD28, point mutants, translocations GOF23,41  10-1523,41,43,44  
 DNMT3A, point mutants LOF12,52  3-3312,52,83  
 TET2, nonsense/point mutants LOF12  13-7312,52,82,83  
 IDH2, point mutants Neomorphic97  20-4012,52,97  
ALK ALCL   
 PTPN6, aberrant methylation and inactivation LOF15,17  5016,17  
 DUSP22, translocations LOF 30 
 VAV1, translocations, point mutants GOF 11 
 JAK1, point mutants GOF54  2154  
 STAT3, point mutants GOF54  1054  
ATLL   
 VAV1, point mutants Likely GOF14  1814  
 PLCG1, point mutants GOF14  3614,75  
 PRKCB, point mutants GOF14  3314,110  
 CARD11, point mutants, CNV (gain) GOF14  2414,75  
 CD28, point mutants, CNV (gain), translocations GOF14  2014,75  
 CCR4, nonsense/point mutants GOF14  2814,75  
 PTPRM, point mutants LOF14  1214  
 STAT3, point mutations GOF14  2214,75  
 RHOA, point mutants GOF14 /unknown29  814  
CTCL   
 PLCG1, point mutants (often S345F), CNV (gain) GOF24,35,36  3-2024,35-37  
 CARD11, CNV (gain), point mutants GOF24,36  2924,36  
 ZEB1, CNV (loss) GOF36  6036  
 JAK2, CNV (gain) GOF24  1336  
 STAT3, CNV (gain), point mutants GOF24,36  6124  
 STAT5B, CNV (gain), point mutants GOF24,36  10-6224,36  
 DNMT3A, CNV (loss) LOF24  4-37.524,36  
 SETD2, CNV (loss) GOF24  2824  
 ARID1A, CNV (loss) LOF24,36  59-6324,36  
 NCOR1, CNV (loss) LOF36  8324  
 RLTPR, point mutant (Q575E) GOF24  724  
EATL/NKTCL   
 STAT5B, point mutants, indels GOF34  2834  
 JAK1, point mutants GOF34  2334  
 JAK3, point mutants GOF34  2334  
 SETD2, point/nonsense mutants LOF34  3234  
 BCOR, CNV (loss), point mutants LOF85  3285  
HSTCL   
 STAT5B, point mutants GOF68  3068  
 STAT3, point mutants, indels GOF68  968  
 SETD2, nonsense/point mutants LOF68  2568  
 TET3, point mutants LOF68  1568  
PTCL NOS   
 RHOA, point mutants LOF12 /unknown23,29  12.5-4012,14,23,28  
 VAV1, translocation, point mutants GOF7,28  11-157,28  
 DNMT3A, point mutants LOF12  1212  
 TET2, nonsense/point mutants LOF12,25  11-3012,25  
SS   
 PLCG1, point mutants (often S345F), indels GOF38  1138  
 ZEB1, point/nonsense mutants LOF38,39  11-5538,39  
 TET1, CNV (loss), point mutants LOF38  5038  
 DNMT3A, CNV (loss), point mutants LOF38  1838  
 KMT2C, nonsense mutants LOF38  3238  
 KMT2B, nonsense mutants, CNV (loss) LOF38  2238  
 ARID1a, CNV (loss), point mutants LOF38,39  33-4138,39  
 NCOR1, CNV (loss), nonsense/point mutants LOF38  5138  
Gene, mutationChange in activityFrequency, %
AITL   
 RHOA, G17V LOF12,31 /unknown29  42-6712,23,28,52,82  
 CD28, point mutants, translocations GOF23,41  10-1523,41,43,44  
 DNMT3A, point mutants LOF12,52  3-3312,52,83  
 TET2, nonsense/point mutants LOF12  13-7312,52,82,83  
 IDH2, point mutants Neomorphic97  20-4012,52,97  
ALK ALCL   
 PTPN6, aberrant methylation and inactivation LOF15,17  5016,17  
 DUSP22, translocations LOF 30 
 VAV1, translocations, point mutants GOF 11 
 JAK1, point mutants GOF54  2154  
 STAT3, point mutants GOF54  1054  
ATLL   
 VAV1, point mutants Likely GOF14  1814  
 PLCG1, point mutants GOF14  3614,75  
 PRKCB, point mutants GOF14  3314,110  
 CARD11, point mutants, CNV (gain) GOF14  2414,75  
 CD28, point mutants, CNV (gain), translocations GOF14  2014,75  
 CCR4, nonsense/point mutants GOF14  2814,75  
 PTPRM, point mutants LOF14  1214  
 STAT3, point mutations GOF14  2214,75  
 RHOA, point mutants GOF14 /unknown29  814  
CTCL   
 PLCG1, point mutants (often S345F), CNV (gain) GOF24,35,36  3-2024,35-37  
 CARD11, CNV (gain), point mutants GOF24,36  2924,36  
 ZEB1, CNV (loss) GOF36  6036  
 JAK2, CNV (gain) GOF24  1336  
 STAT3, CNV (gain), point mutants GOF24,36  6124  
 STAT5B, CNV (gain), point mutants GOF24,36  10-6224,36  
 DNMT3A, CNV (loss) LOF24  4-37.524,36  
 SETD2, CNV (loss) GOF24  2824  
 ARID1A, CNV (loss) LOF24,36  59-6324,36  
 NCOR1, CNV (loss) LOF36  8324  
 RLTPR, point mutant (Q575E) GOF24  724  
EATL/NKTCL   
 STAT5B, point mutants, indels GOF34  2834  
 JAK1, point mutants GOF34  2334  
 JAK3, point mutants GOF34  2334  
 SETD2, point/nonsense mutants LOF34  3234  
 BCOR, CNV (loss), point mutants LOF85  3285  
HSTCL   
 STAT5B, point mutants GOF68  3068  
 STAT3, point mutants, indels GOF68  968  
 SETD2, nonsense/point mutants LOF68  2568  
 TET3, point mutants LOF68  1568  
PTCL NOS   
 RHOA, point mutants LOF12 /unknown23,29  12.5-4012,14,23,28  
 VAV1, translocation, point mutants GOF7,28  11-157,28  
 DNMT3A, point mutants LOF12  1212  
 TET2, nonsense/point mutants LOF12,25  11-3012,25  
SS   
 PLCG1, point mutants (often S345F), indels GOF38  1138  
 ZEB1, point/nonsense mutants LOF38,39  11-5538,39  
 TET1, CNV (loss), point mutants LOF38  5038  
 DNMT3A, CNV (loss), point mutants LOF38  1838  
 KMT2C, nonsense mutants LOF38  3238  
 KMT2B, nonsense mutants, CNV (loss) LOF38  2238  
 ARID1a, CNV (loss), point mutants LOF38,39  33-4138,39  
 NCOR1, CNV (loss), nonsense/point mutants LOF38  5138  

AITL, angioimmunoblastic T-cell lymphoma; ATLL, adult T-cell leukemia/lymphoma; CNV, copy number variation; CTCL, cutaneous T-cell lymphoma; EATL, enteropathy-associated T-cell lymphoma; GOF, gain of function; HSTCL, hepatosplenic T-cell lymphoma; LOF, loss of function; NKTCL, natural killer/T-cell lymphoma; NOS, not otherwise specified; SS, Sézary syndrome.

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