Preti A, Kantarjian H M, Estey E, Huh Y, Keating M, Pierce S, Hirsch-Ginsberg C, Yee G, Stass S A
Department of Hematology, University of Texas M.D. Anderson Cancer Center Houston 77030.
Hematol Pathol. 1994;8(4):155-67.
The purpose of the study was to analyze the clinical and laboratory characteristics of patients with acute lymphocytic leukemia (ALL) who exhibited myeloperoxidase-positive blasts by electron microscopy (EM-MPO-positive), and assess their response to therapy and their prognosis. Since 1988, 21 adults with newly-diagnosed ALL and EM-MPO-positive blasts were referred to our service. In addition to documentation of their clinical and hematopathologic characteristics, patients underwent cytogenetic, immunophenotypic, molecular, and electron-microscopic evaluations. Twenty patients were treated with the vincristine-Adriamycin-dexamethasone (VAD) regimen, and one patient was induced with amsacrine and high-dose cytosine arabinoside (ara-C). The 21 patients were among 141 patients with ALL (15%) seen during the same period. Their median age was 46 years (range 15 to 77 years). The immunophenotype was T-cell ALL in 12 patients (57%). Karyotypic studies did not demonstrate specific recurrent abnormalities. The median percentage of EM-MPO-positive blasts was 15% (range 3% to 45%). Eighteen patients (85%) had high-risk ALL. With induction chemotherapy 15 of 20 (75%) receiving VAD therapy achieved a complete remission (CR). However, the median CR duration was 18 months, and the median survival was 18 months with a 3-year disease-free survival rate of 25%. There were eight relapses and one lineage switch to acute myelogenous leukemia (AML). Patients with ALL and EM-MPO-positive disease are a unique subgroup with long-term poor prognosis on conventional anti-ALL therapy, and may benefit from intensification treatments with agents effective against AML.
本研究的目的是分析经电子显微镜检查显示髓过氧化物酶阳性原始细胞(EM-MPO阳性)的急性淋巴细胞白血病(ALL)患者的临床和实验室特征,并评估他们对治疗的反应及其预后。自1988年以来,21例新诊断为ALL且原始细胞为EM-MPO阳性的成年患者转诊至我院。除记录其临床和血液病理学特征外,患者还接受了细胞遗传学、免疫表型、分子和电子显微镜评估。20例患者接受长春新碱-阿霉素-地塞米松(VAD)方案治疗,1例患者采用安吖啶和大剂量阿糖胞苷(ara-C)诱导治疗。这21例患者是同期所见的141例ALL患者中的一部分(15%)。他们的中位年龄为46岁(范围15至77岁)。免疫表型为T细胞ALL的有12例患者(57%)。核型研究未显示特定的复发性异常。EM-MPO阳性原始细胞的中位百分比为15%(范围3%至45%)。18例患者(85%)患有高危ALL。接受VAD治疗的20例患者中有15例(75%)通过诱导化疗实现了完全缓解(CR)。然而,CR的中位持续时间为18个月,中位生存期为18个月,3年无病生存率为25%。有8例复发,1例发生谱系转换为急性髓系白血病(AML)。ALL且患有EM-MPO阳性疾病的患者是一个独特的亚组,对传统抗ALL治疗的长期预后较差,可能受益于使用对AML有效的药物进行强化治疗。