Preti H A, O'Brien S, Giralt S, Beran M, Pierce S, Kantarjian H M
Department of Hematology, University of Texas, M. D. Anderson Cancer Center, Houston 77030.
Am J Med. 1994 Jul;97(1):60-5. doi: 10.1016/0002-9343(94)90049-3.
Philadelphia-Chromosome-positive (Ph-positive) acute lymphocytic leukemia (ALL) is associated with poor outcome in children as well as in adults. We report our experience in patients with Ph-positive ALL and review their clinico-laboratory characteristics, response to different therapies, and overall prognosis.
Since 1980, 41 newly diagnosed patients with Ph-positive ALL were referred to our service. In addition to confirmation of their diagnosis by morphologic studies of bone marrow aspiration and biopsy specimens, patients underwent cytogenetic, immunophenotypic, and molecular studies. Thirty-five patients received vincristine-Adriamycin-dexamethasone (VAD) or cyclophosphamide (CVAD) induction regimens, and 6 patients were treated with other combinations. Thirty-seven patients received salvage therapy that included VAD, high-dose cytosine arabinoside (ara-C)-containing regimens, methotrexate-asparaginase, and high-dose chemotherapy with autologous or allogeneic bone marrow transplantation (BMT).
The 41 patients were among 334 patients with ALL (12%) seen during that same period. Patients with Ph-positive ALL were older and had a significantly lower incidence of anemia and a higher incidence of peripheral leukocytosis, FAB L2 (French-American-British) morphology, common acute lymphocytic leukemia antigens (CALLA), and CD34 marker positivity. With induction chemotherapy, 23 of 41 Ph-positive ALL patients (56%) achieved complete remission, and their median survival and remission duration were 11 months and 9 months, respectively. Thirteen of 31 Ph-positive ALL patients (42%) achieved complete response with high-dose ara-C regimens during salvage therapy.
In our experience, patients with Ph-positive ALL are usually older, have FAB L2 morphology, and are CALLA-positive and CD34-positive. These patients have a poor prognosis when treated with conventional approaches with lower overall complete response rate, shorter remission duration, and shorter survival. There appears to be a selective sensitivity to high-dose ara-C in these patients that suggests a possible role of this agent as part of early consolidation or induction regimens.
费城染色体阳性（Ph阳性）急性淋巴细胞白血病（ALL）在儿童和成人中均与不良预后相关。我们报告了我们对Ph阳性ALL患者的治疗经验，并回顾了他们的临床实验室特征、对不同治疗方法的反应以及总体预后。
自1980年以来，41例新诊断的Ph阳性ALL患者转诊至我院。除通过骨髓穿刺和活检标本的形态学研究确诊外，患者还接受了细胞遗传学、免疫表型和分子研究。35例患者接受长春新碱-阿霉素-地塞米松（VAD）或环磷酰胺（CVAD）诱导方案治疗，6例患者接受其他联合治疗。37例患者接受挽救治疗，包括VAD、含大剂量阿糖胞苷（ara-C）的方案、甲氨蝶呤-天冬酰胺酶以及自体或异基因骨髓移植（BMT）的大剂量化疗。
这41例患者是同期334例ALL患者中的一部分（占12%）。Ph阳性ALL患者年龄较大，贫血发生率显著较低，外周血白细胞增多、FAB L2（法美英）形态、常见急性淋巴细胞白血病抗原（CALLA）以及CD34标记阳性的发生率较高。诱导化疗后，41例Ph阳性ALL患者中有23例（56%）达到完全缓解，他们的中位生存期和缓解期分别为11个月和9个月。31例Ph阳性ALL患者中有13例（42%）在挽救治疗期间通过大剂量ara-C方案达到完全缓解。
根据我们的经验，Ph阳性ALL患者通常年龄较大，具有FAB L2形态，且CALLA阳性和CD34阳性。这些患者采用传统方法治疗时预后较差，总体完全缓解率较低，缓解期较短，生存期较短。这些患者似乎对大剂量ara-C有选择性敏感性，这表明该药物作为早期巩固或诱导方案的一部分可能发挥作用。
