Table 3. Summary of select studies of... | American Society of Hematology

Table 3.

Summary of select studies of PTCL-NOS in pediatric patients

Clinical stage and number of cases	Initial treatment approach	Study findings	Survival	Reference
Stage I/II, n = 6 Stage III/IV, n = 6	CHOP for stage I/II (n = 6) APO w/wo IDMTX and HiDAC for stage III/IV (n = 6)	No relapses in patients with limited stage disease Relapse occurred in 4/6 with advanced stage	5-y EFS: 60%∗, 5-y OS: 70%∗	6
Stage I/II, n = 7 Stage III/IV, n = 10	T-LLy/ALL regimen (n = 12) B-NHL regimen (n = 5)	5/7 with stage I/II survived 5/10 with stage III/IV disease survived	Follow-up: 59% were alive	7
Stage I/II, n = 3 Stage III/IV, n = 15	LLy regimen (n = 5) ALCL-like regimen (n = 12) B-NHL regimen (n = 1)	16 achieved CR 5 relapsed after reaching CR; 1 survived after relapse	5-y EFS and OS: 61% ± 11% and 65% ± 11%	9
Stage I/II, n = 14 Stage III/IV, n = 46	B-NHL regimen (n = 29) T-LLy regimen (n = 22) Other approach (n = 6) No chemotherapy (n = 3)	No significant survival difference between B-NHL vs T-LLy regimens Relapse in 33% (20) with median time 8.5 mo Progressive disease in 10 6 received HSCT in CR1	5-y EFS and OS: 47% ± 7% and 56% ± 7%	10
Stage I/II, n = 0 Stage III/IV, n = 7	B-NHL regimen (n = 1) APO regimen (n = 2) T-ALL regimen (n = 2) Other approach (n = 1) No chemotherapy (n = 1)	1 achieved CR with chemotherapy only 1 received HSCT in CR1 4 with relapsed/refractory disease (3 received HSCT after relapse)	Alive in CR1: n = 2 Total alive: n = 4	²⁵

Clinical stage and number of cases	Initial treatment approach	Study findings	Survival	Reference
Stage I/II, n = 6 Stage III/IV, n = 6	CHOP for stage I/II (n = 6) APO w/wo IDMTX and HiDAC for stage III/IV (n = 6)	No relapses in patients with limited stage disease Relapse occurred in 4/6 with advanced stage	5-y EFS: 60%∗, 5-y OS: 70%∗	6
Stage I/II, n = 7 Stage III/IV, n = 10	T-LLy/ALL regimen (n = 12) B-NHL regimen (n = 5)	5/7 with stage I/II survived 5/10 with stage III/IV disease survived	Follow-up: 59% were alive	7
Stage I/II, n = 3 Stage III/IV, n = 15	LLy regimen (n = 5) ALCL-like regimen (n = 12) B-NHL regimen (n = 1)	16 achieved CR 5 relapsed after reaching CR; 1 survived after relapse	5-y EFS and OS: 61% ± 11% and 65% ± 11%	9
Stage I/II, n = 14 Stage III/IV, n = 46	B-NHL regimen (n = 29) T-LLy regimen (n = 22) Other approach (n = 6) No chemotherapy (n = 3)	No significant survival difference between B-NHL vs T-LLy regimens Relapse in 33% (20) with median time 8.5 mo Progressive disease in 10 6 received HSCT in CR1	5-y EFS and OS: 47% ± 7% and 56% ± 7%	10
Stage I/II, n = 0 Stage III/IV, n = 7	B-NHL regimen (n = 1) APO regimen (n = 2) T-ALL regimen (n = 2) Other approach (n = 1) No chemotherapy (n = 1)	1 achieved CR with chemotherapy only 1 received HSCT in CR1 4 with relapsed/refractory disease (3 received HSCT after relapse)	Alive in CR1: n = 2 Total alive: n = 4	²⁵

ALL, acute lymphoblastic leukemia; APO, doxorubicin, vincristine, prednisone, mercaptopurine, and methotrexate; B-NHL, mature B-cell NHL; CHOP, cyclophosphamide, doxorubicin, vincristine and prednisolone; EFS, event-free survival; HiDAC, high-dose cytarabine; IDMTX, intermediate-dose methotrexate; T-LLy, T-cell lymphoblastic lymphoma; w/wo, with/without.

∗

Indicates survival estimates for entire cohort of mature T-cell lymphomas, including PTCL-NOS.

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