Philadelphia chromosome positive childhood acute lymphoblastic leukemia: clinical and cytogenetic characteristics and treatment outcome. A Pediatric Oncology Group study
Philadelphia chromosome positive childhood acute lymphoblastic leukemia: clinical and cytogenetic characteristics and treatment outcome. A Pediatric Oncology Group study
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费城染色体阳性儿童急性淋巴细胞白血病：临床和细胞遗传学特征以及治疗结果。
DOI：
10.1182/blood.v76.3.489.bloodjournal763489
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发表时间：
1990
期刊：
影响因子：
20.3
通讯作者：
A. Ragab
中科院分区：
文献类型：
--
作者：
W. Crist;A. Carroll;J. Shuster;J. Jackson;D. Head;M. Borowitz;F. Behm;M. Link;P. Steuber;A. Ragab
在1981年6月至1989年4月期间进入儿科肿瘤组（POG）方案的3，638例急性淋巴细胞白血病（ALL）儿童中，2，519例成功进行了细胞遗传学研究，其中58例（2.3%）检测到费城（Ph）染色体。与Ph染色体存在相关的特征是高白细胞计数（中位数为33 × 109/L），年龄中位数为9.6岁），法裔美国人-英国人L2形态比例较高，纵隔肿块发生率较低。在56例Ph+病例中，诊断时的免疫标记物研究确定42例（75%）为早期前B ALL，9例（16%）为前B细胞，5例（9%）为T细胞。该分布与Ph+ ALL中的分布相似。在符合条件的Ph+患者中，强化多药化疗仅诱导了78%的完全缓解，而在没有确定Ph染色体的患者中，这一比例为96%（P <0.001）。在接受POG一线方案治疗的44例符合条件的Ph+患者中，27例治疗失败，相比之下，1，892例未确定Ph染色体的患者中有520例治疗失败（logrank P小于.001）。Ph+ ALL是急性白血病的一种侵袭性形式，常见于年龄较大的儿童，具有高白细胞计数、FAB L2形态和假二倍体核型，并在早期出现多药耐药。因此，Ph+病例需要早期识别，以便采用强化诱导方案和实验方法（如骨髓移植）进行治疗。
Among 3,638 children with acute lymphoblastic leukemia (ALL) entered on Pediatric Oncology Group (POG) protocols between June 1981 and April 1989, successful cytogenetic studies were available for 2,519, 58 (2.3%) of which had the Philadelphia (Ph) chromosome detected. Features associated with the presence of the Ph chromosome were high leukocyte count (median, 33 x 10(9)/L), older age median, 9.6 years), a higher proportion of French-American-British L2 morphology, and a lower frequency of mediastinal mass. Immunologic marker studies at diagnosis in 56 Ph+ cases identified early pre-B ALL in 42 cases (75%), pre-B-cell in 9 (16%), and T-cell in 5 (9%). This distribution is similar to that found in Ph+ ALL. Intensive multiagent chemotherapy induced complete remissions in only 78% of eligible Ph+ patients compared with 96% of those without an identified Ph chromosome (P less than .001). Of 44 eligible Ph+ patients treated on POG frontline protocols for children with non-T, non-B-cell ALL, 27 have failed therapy, compared with 520 of 1,892 without an identified Ph chromosome (logrank P less than .001). Ph+ ALL is an aggressive form of acute leukemia that frequently presents in older children with a high leukocyte count, FAB L2 morphology, and a pseudodiploid karyotype, and becomes multidrug-resistant early. Thus, Ph+ cases require early identification to permit treatment with intensive induction regimens and experimental approaches such as bone marrow transplantation.
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影响因子：
20.3
作者：
W. Crist;J. Boyett;J. Jackson;T. Vietti;M. Borowitz;A. Chauvenet;N. Winick;A. Ragab;Donald H. Mahoney;D. Head;R. Iyer;H. Wagner;J. Pullen
通讯作者：
W. Crist;J. Boyett;J. Jackson;T. Vietti;M. Borowitz;A. Chauvenet;N. Winick;A. Ragab;Donald H. Mahoney;D. Head;R. Iyer;H. Wagner;J. Pullen
结构染色体异常对超二倍体（超过 50 条染色体）急性淋巴细胞白血病儿童的预后重要性。
影响因子：
20.3
作者：
Pui,CH;Raimondi,SC;Dodge,RK;Rivera,GK;Fuchs,LA;Abromowitch,M;Look,AT;Furman,WL;Crist,WM;Williams,DL
通讯作者：
Williams,DL
细胞遗传学研究对急性淋巴细胞白血病儿童强化治疗组的预后影响。
影响因子：
20.3
作者：
Fletcher,JA;Kimball,VM;Lynch,E;Donnelly,M;Pavelka,K;Gelber,RD;Tantravahi,R;Sallan,SE
通讯作者：
Sallan,SE
根据胸腺细胞分化阶段的儿童 T 细胞白血病-淋巴瘤的临床特征和结果：一项儿科肿瘤学小组研究。
影响因子：
20.3
作者：
Crist,WM;Shuster,JJ;Falletta,J;Pullen,DJ;Berard,CW;Vietti,TJ;Alvarado,CS;Roper,MA;Prasthofer,E;Grossi,CE
通讯作者：
Grossi,CE
儿童急性淋巴细胞白血病费城染色体的临床和生物学特征。
影响因子：
20.3
作者：
Ribeiro,RC;Abromowitch,M;Raimondi,SC;Murphy,SB;Behm,F;Williams,DL
通讯作者：
Williams,DL