Immunological Phenotype and Prognosis of T-Lineage Malignancy in Children

The efficacy of high-dose cytosine arabinoside (HDCA) and phenotypic difference in the prognosis were investigated in T-lineage malignancies (ALL and NHL) in children. Twenty-nine patients from July 1984 to December 1991 were included in this study. Ten patients were treated with the regimen without...

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Published inThe Japanese Journal of Pediatric Hematology Vol. 7; no. 1; pp. 41 - 46
Main Authors INOUE, Masami, HARA, Junichi, SAKATA, Naoki, TAWA, Akio, KAW-HA, Keisei, ISHIHARA, Shigehiko, OSUGI, Yuko, KOUDERA, Urara, KURAHASHI, Hiroki, YUMURA-YAGI, Keiko, KONISHI, Syouzaburou, SAKO, Masanori
Format Journal Article
LanguageJapanese
Published THE JAPANESE SOCIETY OF PEDIATRIC HEMATOLOGY/ONCOLOGY 1993
特定非営利活動法人 日本小児血液・がん学会
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ISSN0913-8706
1884-4723
DOI10.11412/jjph1987.7.41

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Abstract The efficacy of high-dose cytosine arabinoside (HDCA) and phenotypic difference in the prognosis were investigated in T-lineage malignancies (ALL and NHL) in children. Twenty-nine patients from July 1984 to December 1991 were included in this study. Ten patients were treated with the regimen without HDCA (Group A), 9 received single HDCA intensification (Group B) and 10 were treated with cyclic HDCA intensifications (Group C). Disease-free survival (DFS) rates at 48 months were 48%, 38.1% and 71%, respectively. Although these values were not significantly different, cyclic HDCA intensifications seem to be effective for T-ALL and NHL in children, because only 2 patients of Group C relapsed. The phenotype of the samples from Group B and C was divided into 3 groups, i.e., CD3 + group; CD3-, CD4+ and/or CD8+group; and CD3-, CD4-and CD8-group. The patients whose leukemic cells expressed CD3 had a favorable prognosis, and the patients with CD3-, CD4-and CD8-had the worst. Further study is needed to clarify whether the phenotype remains a prognostic factor in patients treated with very intensive therapies or not.
AbstractList The efficacy of high-dose cytosine arabinoside (HDCA) and phenotypic difference in the prognosis were investigated in T-lineage malignancies (ALL and NHL) in children. Twenty-nine patients from July 1984 to December 1991 were included in this study. Ten patients were treated with the regimen without HDCA (Group A), 9 received single HDCA intensification (Group B) and 10 were treated with cyclic HDCA intensifications (Group C). Disease-free survival (DFS) rates at 48 months were 48%, 38.1% and 71%, respectively. Although these values were not significantly different, cyclic HDCA intensifications seem to be effective for T-ALL and NHL in children, because only 2 patients of Group C relapsed. The phenotype of the samples from Group B and C was divided into 3 groups, i.e., CD3 + group; CD3-, CD4+ and/or CD8+group; and CD3-, CD4-and CD8-group. The patients whose leukemic cells expressed CD3 had a favorable prognosis, and the patients with CD3-, CD4-and CD8-had the worst. Further study is needed to clarify whether the phenotype remains a prognostic factor in patients treated with very intensive therapies or not. 1984年7月から1991年12月までにT系ALLあるいはNHLと診断した29例を対象として, キロサイド大量療法 (HDCA) の有用性とphenotypeと予後の関連について検討した.HDCAを含まないプロトコールで治療された群 (A群) が10例, HDCAの強化療法を1回受けた群 (B群) が9例, HDCA強化療法を定期的に施行された群 (C群) が10例であった.各群の48カ月の無病生存率は各々48%, 38.1%, 70%であり, 有意差は認められなかった.しかし, C群では再発はわずか2例であり, HDCAの定期的な強化療法はT系ALL/NHLに有用であると思われた.B+C群で, phenotypeからCD3, CD4, CD8全て陰性例, CD3陰性でCD4あるいはCD8陽性例, CD3陽性例の3群にわけて予後を検討した.CD3陽性例が最も予後良好で, CD3, CD4, CD8陰性例は予後不良であった (p<0.05).Phenotypeが独立予後因子となりうるかどうかを明らかにするためには, 今後C群のみでのphenotypeと予後の検討が必要であろう.
The efficacy of high-dose cytosine arabinoside (HDCA) and phenotypic difference in the prognosis were investigated in T-lineage malignancies (ALL and NHL) in children. Twenty-nine patients from July 1984 to December 1991 were included in this study. Ten patients were treated with the regimen without HDCA (Group A), 9 received single HDCA intensification (Group B) and 10 were treated with cyclic HDCA intensifications (Group C). Disease-free survival (DFS) rates at 48 months were 48%, 38.1% and 71%, respectively. Although these values were not significantly different, cyclic HDCA intensifications seem to be effective for T-ALL and NHL in children, because only 2 patients of Group C relapsed. The phenotype of the samples from Group B and C was divided into 3 groups, i.e., CD3 + group; CD3-, CD4+ and/or CD8+group; and CD3-, CD4-and CD8-group. The patients whose leukemic cells expressed CD3 had a favorable prognosis, and the patients with CD3-, CD4-and CD8-had the worst. Further study is needed to clarify whether the phenotype remains a prognostic factor in patients treated with very intensive therapies or not.
Author TAWA, Akio
KAW-HA, Keisei
ISHIHARA, Shigehiko
KURAHASHI, Hiroki
HARA, Junichi
SAKATA, Naoki
KOUDERA, Urara
SAKO, Masanori
YUMURA-YAGI, Keiko
INOUE, Masami
OSUGI, Yuko
KONISHI, Syouzaburou
Author_FL 河 敬世
多和 昭雄
圀府寺 美
倉橋 浩樹
大杉 夕子
坂田 尚己
小西 省三郎
迫 正廣
井上 雅美
勇村 啓子
原 純一
石原 重彦
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  fullname: SAKO, Masanori
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特定非営利活動法人 日本小児血液・がん学会
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References 9) Dahl GV, Rivera GK, Look AT, et al : Teniposide plus cytarabine improved outcome in childhood acute lymphoblastic leukemia presenting with a leukocyte count greater than or equal to 100,000/mm3. J Clin Oncol 5 : 1015-1021, 1987
2) Weinstein HJ, Cassady R, Levey R : Long-term results of the APO protocol (vincristine, doxorubicin [adriamycin], and prednisone) for treatment of mediastinal lymphoblastic lymphoma. J Clin Oncol 1 : 537-541, 1983
13) Yumura-Yagi K, Hara J, Kurahashi H, et al : Clinical significance of CD7-positive stem cell leukmia; a distinct subgroup of mixed lineage leukemia. Cancer 68 : 2273-2280, 1991
5) Yumura-Yagi K, Ishihara S, Hara J, et al : Poor prognosis of mediastinal non Hodgkin's lymphoma with an immature phenotype of CD2+, CD7 (or CD5) +, CD3-, CD4-, and CD8 -. Cancer 63 : 671-674, 1989
1) Sullivan MP, Boyett J, Pullen J, et al : Pediatric oncology group experience with modified LSA2-L2 therapy in 107 children with non Hodgkin's lymphoma (Burkitt's lymphoma excluded). Cancer 55 : 323-336, 1985
6) Crist WM, Shueter JJ, Falleta J, et al : Clinical features and outcome in childhood T-cell leukemialymphoma according to stage of thymocyte differentiation. : a pediatric oncology group study. Blood 72 : 1891-1897, 1988
15) Pullen DJ, Sullivan MP, Falletta JM, et al : Modified LSA2L2 treatment in 53 children with E-rosette positive T-cell leukemia : results and prognostic factors. Blood 60 : 1159, 1982
14) Yumura-Yagi K, Hara J, Terada N, et al : Analysis of molecular events in leukemic cells arrested at early stage of T-cell differentiation. Blood 74 : 2103-2111, 1989
7) Amylon MD : Treatment of T-lineage acute lymphoblastic leukemia. Hematol/Oncol Clin North Am 4 : 937-949, 1991
12) Hara J, Yumura-Yagi K, Tawa A, et al : Molecular analysis of acute undifferentiated leukemia : two distinct subgroups at the DNA and RNA levels. Blood 74 : 1738-1746, 1989
16) Pui C-H, Ribeiro RC, Hancock MS, et al : Acute myeloid leukemia in children treated with epipodophyllotoxins for acute lymphoblastic leukemia. N Engl J Med 325 : 1682-1687, 1991
8) Weily JS, Traupin J, Jamieson GP, et al : Cytosine arabinoside transport and T-cell lymphoblastic lymphoma. J Clin Invest 75 : 632-642, 1985
10) 勇村啓子, 河敬世, 石原重彦, 他 : 小児悪性リンパ腫 (Non-Hodgkin's Lymphoma) に対するAra-C大量療法-強化療法ならびにCNS予防としての有用性-.臨床血液 28 : 371-376, 1987
11) Shuster JJ, Falletta LH, Pullen L, et al : Prognostic factor in T-cell acute lymphoblastic leukemia : a pediatric oncology group study. Blood 75 : 166-173, 1990
3) Lauer SJ, Pinkel D, Buchanan GR, et al : Cytosine arabinoside/cyclophosphamide pulses during continuation therapy for childhood acute lymphoblastic leukemia; potential selective effect in T-cell leukemia. Cancer 60 : 2366-2371, 1987
4) Pui C-H, Behm FG, Singh B, et al : Heterogeneity of presenting features and their relation to treatment outcome in 120 children with T-cell acute lymphoblastic leukemia. Blood 75 : 174-179, 1990
References_xml – reference: 6) Crist WM, Shueter JJ, Falleta J, et al : Clinical features and outcome in childhood T-cell leukemialymphoma according to stage of thymocyte differentiation. : a pediatric oncology group study. Blood 72 : 1891-1897, 1988
– reference: 2) Weinstein HJ, Cassady R, Levey R : Long-term results of the APO protocol (vincristine, doxorubicin [adriamycin], and prednisone) for treatment of mediastinal lymphoblastic lymphoma. J Clin Oncol 1 : 537-541, 1983
– reference: 16) Pui C-H, Ribeiro RC, Hancock MS, et al : Acute myeloid leukemia in children treated with epipodophyllotoxins for acute lymphoblastic leukemia. N Engl J Med 325 : 1682-1687, 1991
– reference: 13) Yumura-Yagi K, Hara J, Kurahashi H, et al : Clinical significance of CD7-positive stem cell leukmia; a distinct subgroup of mixed lineage leukemia. Cancer 68 : 2273-2280, 1991
– reference: 1) Sullivan MP, Boyett J, Pullen J, et al : Pediatric oncology group experience with modified LSA2-L2 therapy in 107 children with non Hodgkin's lymphoma (Burkitt's lymphoma excluded). Cancer 55 : 323-336, 1985
– reference: 4) Pui C-H, Behm FG, Singh B, et al : Heterogeneity of presenting features and their relation to treatment outcome in 120 children with T-cell acute lymphoblastic leukemia. Blood 75 : 174-179, 1990
– reference: 3) Lauer SJ, Pinkel D, Buchanan GR, et al : Cytosine arabinoside/cyclophosphamide pulses during continuation therapy for childhood acute lymphoblastic leukemia; potential selective effect in T-cell leukemia. Cancer 60 : 2366-2371, 1987
– reference: 8) Weily JS, Traupin J, Jamieson GP, et al : Cytosine arabinoside transport and T-cell lymphoblastic lymphoma. J Clin Invest 75 : 632-642, 1985
– reference: 15) Pullen DJ, Sullivan MP, Falletta JM, et al : Modified LSA2L2 treatment in 53 children with E-rosette positive T-cell leukemia : results and prognostic factors. Blood 60 : 1159, 1982
– reference: 14) Yumura-Yagi K, Hara J, Terada N, et al : Analysis of molecular events in leukemic cells arrested at early stage of T-cell differentiation. Blood 74 : 2103-2111, 1989
– reference: 7) Amylon MD : Treatment of T-lineage acute lymphoblastic leukemia. Hematol/Oncol Clin North Am 4 : 937-949, 1991
– reference: 9) Dahl GV, Rivera GK, Look AT, et al : Teniposide plus cytarabine improved outcome in childhood acute lymphoblastic leukemia presenting with a leukocyte count greater than or equal to 100,000/mm3. J Clin Oncol 5 : 1015-1021, 1987
– reference: 10) 勇村啓子, 河敬世, 石原重彦, 他 : 小児悪性リンパ腫 (Non-Hodgkin's Lymphoma) に対するAra-C大量療法-強化療法ならびにCNS予防としての有用性-.臨床血液 28 : 371-376, 1987
– reference: 12) Hara J, Yumura-Yagi K, Tawa A, et al : Molecular analysis of acute undifferentiated leukemia : two distinct subgroups at the DNA and RNA levels. Blood 74 : 1738-1746, 1989
– reference: 5) Yumura-Yagi K, Ishihara S, Hara J, et al : Poor prognosis of mediastinal non Hodgkin's lymphoma with an immature phenotype of CD2+, CD7 (or CD5) +, CD3-, CD4-, and CD8 -. Cancer 63 : 671-674, 1989
– reference: 11) Shuster JJ, Falletta LH, Pullen L, et al : Prognostic factor in T-cell acute lymphoblastic leukemia : a pediatric oncology group study. Blood 75 : 166-173, 1990
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phenotype
T-lineage ALL/NHL
Title Immunological Phenotype and Prognosis of T-Lineage Malignancy in Children
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