Gustafson Steven A, Lin Pei, Chen Su S, Chen Lei, Abruzzo Lynne V, Luthra Rajyalakshmi, Medeiros L Jeffrey, Wang Sa A
Department of Hematopathology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA.
Am J Clin Pathol. 2009 May;131(5):647-55. doi: 10.1309/AJCP5ETHDXO6NCGZ.
To determine if therapy-related acute myeloid leukemia (t-AML) with t(8;21)(q22;q22) [t-AML-t(8;21)] harbors similar characteristic clinicopathologic features as de novo AML-t(8;21) (q22;q22), we studied 13 cases of t-AML-t(8;21) and 38 adult cases of de novo AML-t(8;21) diagnosed and treated at our hospital (1995-2008). Of 13 t-AML-t(8;21) cases, 11 had previously received chemotherapy with or without radiation for malignant neoplasms and 2 received radiation alone. The median latency to t-AML onset was 37 months (range, 11-126 months). Compared with patients with de novo AML-t(8;21), patients with t-AML-t(8;21) were older (P = .001) and had a lower WBC count (P = .039), substantial morphologic dysplasia, and comparable CD19/CD56 expression. The AML1-ETO (RUNX1-RUNX1T1) fusion was demonstrated in all 10 cases assessed. Class I mutations analyzed included FLT3 (0/10 [0%]), RAS (0/10 [0%]), JAK2 V617 (0/11 [0%]), and KIT (4/11 [36%]). With a median follow-up of 13 months, 10 patients with t-AML-t(8;21) died; the overall survival was significantly inferior to that of patients with de novo AML-t(8;21) (19 months vs not reached; P = .002). These findings suggest that t-AML-t(8;21) shares many features with de novo AML-t(8;21)(q22;q22), but affected patients have a worse outcome.
为确定伴t(8;21)(q22;q22)的治疗相关急性髓系白血病(t-AML)[t-AML-t(8;21)]是否具有与原发性AML-t(8;21)(q22;q22)相似的特征性临床病理特征,我们研究了在我院(1995 - 2008年)诊断和治疗的13例t-AML-t(8;21)及38例成人原发性AML-t(8;21)。13例t-AML-t(8;21)病例中,11例曾接受过针对恶性肿瘤的化疗(伴或不伴放疗),2例仅接受过放疗。t-AML发病的中位潜伏期为37个月(范围11 - 126个月)。与原发性AML-t(8;21)患者相比,t-AML-t(8;21)患者年龄更大(P = 0.001),白细胞计数更低(P = 0.039),有明显的形态学发育异常,且CD19/CD56表达相当。在所有评估的10例病例中均检测到AML1-ETO(RUNX1-RUNX1T1)融合。分析的I类突变包括FLT3(0/10 [0%])、RAS(0/10 [0%])、JAK2 V617(0/11 [0%])和KIT(4/11 [36%])。中位随访13个月时,10例t-AML-t(8;21)患者死亡;总生存期显著低于原发性AML-t(8;21)患者(19个月对未达到;P = 0.002)。这些发现表明,t-AML-t(8;21)与原发性AML-t(8;2)1)(q22;q22)有许多共同特征,但患病患者的预后更差。