Table 4.

Clinical characteristics of ATLL patients who achieved first CR by regimen

Patient codeATLL subtypeLDH × UNLCalcium level, mg/dLExtranodal involvementWBC/ALC × 109/LRegimen used prior to CROutcome/statusPFS
ATLL-1 Acute 6.3 13.6 PB/liver 13/10 AI as second line (failed CHOP-like/fludarabine) Relapsed (as lymphomatous)/deceased 6.3 y 
ATLL-2 Acute 4.8 9.1 PB/liver 37/31 AI Relapsed (as lymphomatous)/deceased 6.3 y 
ATLL-3 Unf chronic 1.1 9.1 PB 28/23 AI Relapsed/alive 10.6 y 
ATLL-4 Acute* 6.2 8.7 BM/PB/skin 36.6/9.7 AI Sustained response/alive 3.3 y 
ATLL-14 Acute 2.3 9.9 BM/PB 152/115 CHOP after AI Lost follow up 2 mo 
ATLL-21 Unf chronic 2.4 9.6 PB 61/52 AI Relapsed (as lymphomatous) 3.4 y 
ATLL-22 Acute 7.7 12.5 PB 153/141 Etoposide (oral) Relapsed/deceased 4 mo 
ATLL-23 Acute 9.3 PB/GI 1.5/1.3 EPOCH + AI maintenance Relapsed/deceased 6 mo 
ATLL-24 Acute 6.8 12.2 Skin/lung/PB 102/84.7 Etoposide (oral) Relapsed/deceased 5 mo 
ATLL-26 Acute 4.3 9.2 PB 53/47 Etoposide (oral) Relapsed/deceased 14 mo 
ATLL-27 Acute 2.4 10.4 PB/skin 89/84 AI Relapsed/deceased 2.6 y 
ATLL-29 Lymphomatous 9.7 BM/nasopharynx 4.6/1.8 EPOCH + AI maintenance Relapsed/deceased 13 mo 
ATLL-33 Lymphomatous 5.9 10.5 Parotid, CNS 7.9/2.7 Brain XRT followed by high-dose MTX/AZT Relapsed/deceased 7 mo 
ATLL-54 Acute 2.9 13.9 Lung/pleura/pericardium 55/28 AI Relapsed/deceased 3.2 y 
ATLL-55 Acute 14.9 PB/lung/pleura/skin 64/59 CHOP as second-line (after AI) Relapsed/deceased 3 mo 
ATLL-59 Lymphomatous 5.2 2.5 None 5.2/2.5 CHOP Relapsed/deceased 17 mo 
ATLL-60 Acute 4.6 10.3 PB/skin/liver 23/16 AI Lost follow-up/deceased 9.2 mo 
ATLL-66 Lymphomatous 3.2 9.3 Skin 4.0/1.0 AI Relapsed/deceased 2.4 mo 
ATLL-84 Acute 12.1 PB/skin 250 (PP)→40/26 AI Relapsed/deceased 3.8 y 
ATLL-87 Lymphomatous 1.3 12.1 Skin 14/2.9 EPOCH Relapsed/deceased 16 mo 
ATLL-93 Lymphomatous 1.5 9.4 None 3.9/2.1 VCAP-AP-VECP Lost to follow-up/deceased 18 mo 
ATLL-100 Lymphomatous 9.7 Bone 4.9/1.2 EPOCH Relapsed/deceased 14 mo 
ATLL-101 Acute 1.2 17.8 Skin/lung/PB/BM 50/14 CHOP Relapsed/deceased 28 mo 
ATLL-103 Acute 2.5 16.5 Skin/bone/BM/PB 52/38 VCAP-AP-VECP as second-line (after AI) Relapsed/deceased 16 mo 
ATLL-104 Acute 2.8 10.1 Skin/PB 32/23 AI Relapsed/deceased 7 mo 
ATLL-106 Acute Unk 19.6 BM/PB 37/Unk VCAP-AP-VECP Relapsed/deceased 3 mo 
ATLL-107 Acute 2.1 9.7 Skin/lung/bone/pharynx 20/14 AI Relapsed/deceased 4 mo 
ATLL-108 Acute Unk 14 Bone/PB “Elevated” VCAP-AP-VECP Relapsed/deceased 10 mo 
ATLL-110 Acute 1.8 11.1 PB/BM 62/53 AI as 3rd (failed EPOCH 1 cycle, and ICE 1 cycle) Sustained response/alive 6 y 
ATLL-114 Lymphomatous 1.4 13 CNS 7/1.2 High-dose MTX/AZT Relapsed/deceased 7 mo 
ATLL-115 Lymphomatous 10.2 None 4/1.7 EPOCH/bortezomib/ raltegravir Relapsed/deceased 2.3 y 
ATLL-119 Lymphomatous Unk Unk GI/pleura/BM Unk/Unk VCAP-AP-VECP followed by allo-HCT Death from infection during transplant 9 mo 
ATLL-120 Lymphomatous Unk None 6.2/2.1 EPOCH + AZT maintenance Relapsed/deceased 13 mo 
ATLL-123 Lymphomatous 14 Unk Liver/BM Unk/Unk CHOP/CODOX/ICE Relapsed/lost to follow-up 8 mo 
ATLL-126 Acute 4.5 9.6 GI/PB 31/15 VCAP-AP-VECP Relapsed/deceased 7 mo 
ATLL-136 Acute 12.7 12 Bone/PB 57/36 ProMACE-CytaBOM Lost to follow-up/unknown 14 mo 
ATLL-137 Acute Unk Unk Liver/BM/PB 27/13 ProMACE-CytaBOM Relapsed/deceased 18 mo 
ATLL-179 Lymphomatous 3.8 GI 11.7/2 VCAP-AP-VECP followed by allo-HCT Relapsed/deceased 13 mo 
ATLL-180 Lymphomatous 1.4 11 BM Unk/1.4 CHOP Relapsed/deceased 6 mo 
ATLL-181 Lymphomatous 1.3 11.2 None 6.2/2.9 CHOEP Relapsed/alive 14 mo 
ATLL-193 Acute 5.8 14 PB/kidney 89/49 AI Relapsed/deceased 1 mo 
ATLL-194 Lymphomatous Unk Unk BM/GI Unk/Unk CHOP Relapsed/deceased 8 mo 
ATLL-197 Acute 16 Bone/PB/BM 272/245 CHOP Relapsed/deceased 6 mo 
ATLL-200 Acute 3.4 12.3 Liver/PB/BM 45/21 VCAP-AP-VECP as second-line (after AI) Death from sepsis 3 mo 
ATLL-208 Lymphomatous 1.9 8.1 None 5.9/2.9 CHOP Sustained response/alive 21 mo 
ATLL-212 Lymphomatous 7.8 None 2.5/1.4 CHOP Relapsed/deceased 16 mo 
ATLL-214 Lymphomatous 1.1 7.1 GI/liver/lung 4.4/1.2 CHOP Relapsed/deceased 7 mo 
ATLL-215 Acute 8.7 BM/PB 28/12.3 CHOEP followed by allo-HCT Relapsed/deceased after failure to engraft 11 mo 
ATLL-216 Acute 3.4 14 Bone/skin/PB 161/146 AI Sustained response/alive 10 mo 
ATLL-218 Lymphomatous Unk Unk None 4.6/Unk CHOP Relapsed/deceased 8 mo 
ATLL-224 Lymphomatous Unk Unk BM/bone/skin 7.1/1.5 EPOCH Relapsed/alive 6 mo 
Patient codeATLL subtypeLDH × UNLCalcium level, mg/dLExtranodal involvementWBC/ALC × 109/LRegimen used prior to CROutcome/statusPFS
ATLL-1 Acute 6.3 13.6 PB/liver 13/10 AI as second line (failed CHOP-like/fludarabine) Relapsed (as lymphomatous)/deceased 6.3 y 
ATLL-2 Acute 4.8 9.1 PB/liver 37/31 AI Relapsed (as lymphomatous)/deceased 6.3 y 
ATLL-3 Unf chronic 1.1 9.1 PB 28/23 AI Relapsed/alive 10.6 y 
ATLL-4 Acute* 6.2 8.7 BM/PB/skin 36.6/9.7 AI Sustained response/alive 3.3 y 
ATLL-14 Acute 2.3 9.9 BM/PB 152/115 CHOP after AI Lost follow up 2 mo 
ATLL-21 Unf chronic 2.4 9.6 PB 61/52 AI Relapsed (as lymphomatous) 3.4 y 
ATLL-22 Acute 7.7 12.5 PB 153/141 Etoposide (oral) Relapsed/deceased 4 mo 
ATLL-23 Acute 9.3 PB/GI 1.5/1.3 EPOCH + AI maintenance Relapsed/deceased 6 mo 
ATLL-24 Acute 6.8 12.2 Skin/lung/PB 102/84.7 Etoposide (oral) Relapsed/deceased 5 mo 
ATLL-26 Acute 4.3 9.2 PB 53/47 Etoposide (oral) Relapsed/deceased 14 mo 
ATLL-27 Acute 2.4 10.4 PB/skin 89/84 AI Relapsed/deceased 2.6 y 
ATLL-29 Lymphomatous 9.7 BM/nasopharynx 4.6/1.8 EPOCH + AI maintenance Relapsed/deceased 13 mo 
ATLL-33 Lymphomatous 5.9 10.5 Parotid, CNS 7.9/2.7 Brain XRT followed by high-dose MTX/AZT Relapsed/deceased 7 mo 
ATLL-54 Acute 2.9 13.9 Lung/pleura/pericardium 55/28 AI Relapsed/deceased 3.2 y 
ATLL-55 Acute 14.9 PB/lung/pleura/skin 64/59 CHOP as second-line (after AI) Relapsed/deceased 3 mo 
ATLL-59 Lymphomatous 5.2 2.5 None 5.2/2.5 CHOP Relapsed/deceased 17 mo 
ATLL-60 Acute 4.6 10.3 PB/skin/liver 23/16 AI Lost follow-up/deceased 9.2 mo 
ATLL-66 Lymphomatous 3.2 9.3 Skin 4.0/1.0 AI Relapsed/deceased 2.4 mo 
ATLL-84 Acute 12.1 PB/skin 250 (PP)→40/26 AI Relapsed/deceased 3.8 y 
ATLL-87 Lymphomatous 1.3 12.1 Skin 14/2.9 EPOCH Relapsed/deceased 16 mo 
ATLL-93 Lymphomatous 1.5 9.4 None 3.9/2.1 VCAP-AP-VECP Lost to follow-up/deceased 18 mo 
ATLL-100 Lymphomatous 9.7 Bone 4.9/1.2 EPOCH Relapsed/deceased 14 mo 
ATLL-101 Acute 1.2 17.8 Skin/lung/PB/BM 50/14 CHOP Relapsed/deceased 28 mo 
ATLL-103 Acute 2.5 16.5 Skin/bone/BM/PB 52/38 VCAP-AP-VECP as second-line (after AI) Relapsed/deceased 16 mo 
ATLL-104 Acute 2.8 10.1 Skin/PB 32/23 AI Relapsed/deceased 7 mo 
ATLL-106 Acute Unk 19.6 BM/PB 37/Unk VCAP-AP-VECP Relapsed/deceased 3 mo 
ATLL-107 Acute 2.1 9.7 Skin/lung/bone/pharynx 20/14 AI Relapsed/deceased 4 mo 
ATLL-108 Acute Unk 14 Bone/PB “Elevated” VCAP-AP-VECP Relapsed/deceased 10 mo 
ATLL-110 Acute 1.8 11.1 PB/BM 62/53 AI as 3rd (failed EPOCH 1 cycle, and ICE 1 cycle) Sustained response/alive 6 y 
ATLL-114 Lymphomatous 1.4 13 CNS 7/1.2 High-dose MTX/AZT Relapsed/deceased 7 mo 
ATLL-115 Lymphomatous 10.2 None 4/1.7 EPOCH/bortezomib/ raltegravir Relapsed/deceased 2.3 y 
ATLL-119 Lymphomatous Unk Unk GI/pleura/BM Unk/Unk VCAP-AP-VECP followed by allo-HCT Death from infection during transplant 9 mo 
ATLL-120 Lymphomatous Unk None 6.2/2.1 EPOCH + AZT maintenance Relapsed/deceased 13 mo 
ATLL-123 Lymphomatous 14 Unk Liver/BM Unk/Unk CHOP/CODOX/ICE Relapsed/lost to follow-up 8 mo 
ATLL-126 Acute 4.5 9.6 GI/PB 31/15 VCAP-AP-VECP Relapsed/deceased 7 mo 
ATLL-136 Acute 12.7 12 Bone/PB 57/36 ProMACE-CytaBOM Lost to follow-up/unknown 14 mo 
ATLL-137 Acute Unk Unk Liver/BM/PB 27/13 ProMACE-CytaBOM Relapsed/deceased 18 mo 
ATLL-179 Lymphomatous 3.8 GI 11.7/2 VCAP-AP-VECP followed by allo-HCT Relapsed/deceased 13 mo 
ATLL-180 Lymphomatous 1.4 11 BM Unk/1.4 CHOP Relapsed/deceased 6 mo 
ATLL-181 Lymphomatous 1.3 11.2 None 6.2/2.9 CHOEP Relapsed/alive 14 mo 
ATLL-193 Acute 5.8 14 PB/kidney 89/49 AI Relapsed/deceased 1 mo 
ATLL-194 Lymphomatous Unk Unk BM/GI Unk/Unk CHOP Relapsed/deceased 8 mo 
ATLL-197 Acute 16 Bone/PB/BM 272/245 CHOP Relapsed/deceased 6 mo 
ATLL-200 Acute 3.4 12.3 Liver/PB/BM 45/21 VCAP-AP-VECP as second-line (after AI) Death from sepsis 3 mo 
ATLL-208 Lymphomatous 1.9 8.1 None 5.9/2.9 CHOP Sustained response/alive 21 mo 
ATLL-212 Lymphomatous 7.8 None 2.5/1.4 CHOP Relapsed/deceased 16 mo 
ATLL-214 Lymphomatous 1.1 7.1 GI/liver/lung 4.4/1.2 CHOP Relapsed/deceased 7 mo 
ATLL-215 Acute 8.7 BM/PB 28/12.3 CHOEP followed by allo-HCT Relapsed/deceased after failure to engraft 11 mo 
ATLL-216 Acute 3.4 14 Bone/skin/PB 161/146 AI Sustained response/alive 10 mo 
ATLL-218 Lymphomatous Unk Unk None 4.6/Unk CHOP Relapsed/deceased 8 mo 
ATLL-224 Lymphomatous Unk Unk BM/bone/skin 7.1/1.5 EPOCH Relapsed/alive 6 mo 

AI, zidovudine and interferon-α; ALC, absolute lymphocyte count; BM, bone marrow; Ca, calcium; GI, gastrointestinal tract; LN, lymph node; MTX, methotrexate; PB, peripheral blood; PP, plasma exchange; Unf chronic, unfavorable chronic; UNL, upper normal limit; Unk, unknown; WBC, white blood cell count.

*

Patient transformed from unfavorable chronic to acute ATLL prior to AZT-INF.

Patient transformed from smoldering to acute ATLL prior to etoposide.

Censored prior to the transplant conditioning regimen.

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