Suzuki Ritsuro, Murata Makoto, Kami Masahiro, Ohtake Shigeki, Asou Norio, Kodera Yoshihisa, Tomonaga Masao, Masaki Yasufumi, Kusumoto Shuya, Takeuchi Jin, Matsuda Shin, Hirai Hisamaru, Yorimitsu m Seiichi, Hamajima Nobuyuki, Seto Masao, Shimoyama Masanori, Ohno Ryuzo, Morishima Yasuo, Nakamura Shigeo
Division of Molecular Medicine, Aichi Cancer Center, Nagoya, Japan.
Int J Hematol. 2003 Jun;77(5):482-9. doi: 10.1007/BF02986617.
Myeloid/natural killer (NK) cell precursor acute leukemia is an entity of acute leukemia characterized by poor prognosis and a CD7+CD56+ myeloid antigen+ phenotype without light-microscopic myeloperoxidase reactivity. This disease shares several clinical characteristics with acute myeloid leukemia (AML) M0. To clarify the relationship between these 2 leukemias, we analyzed 105 cases of AML M0. Among them, 17 were CD7+ and CD56+, 77 were negative for either antigen, and 11 could not be determined. CD7+CD56+ AML M0 showed onset at significantly lower patient age (median 46 versus 63 years, P = .004). The disease localization and the hematological manifestations were significantly different: CD7+CD56+ AML showed more frequent extramedullary involvement, fewer circulating leukemic blasts, less anemia, and less thrombocytopenia than did AML M0. The cytogenetic aberrations were also unique, because no 5q abnormalities were found in CD7+CD56+ M0. The prognosis of CD7+CD56+ M0 was poor in patients younger than 46 years (P = .03). Multivariate analysis showed that the CD7+CD56+ phenotype was a significant prognostic factor for AML M0, as well as age, circulating blast percentage, and chromosome 5 abnormalities These findings suggest that AML M0 consists of heterogeneous subgroups to be managed separately, and CD7+CD56+ M0 constitutes a distinct subtype of AML M0.
髓系/自然杀伤(NK)细胞前体急性白血病是一种急性白血病实体,其特征为预后不良,具有CD7 + CD56 +髓系抗原+表型,且在光学显微镜下无髓过氧化物酶反应性。该疾病与急性髓系白血病(AML)M0具有若干临床特征。为阐明这两种白血病之间的关系,我们分析了105例AML M0病例。其中,17例为CD7 +和CD56 +,77例对任一抗原呈阴性,11例无法确定。CD7 + CD56 + AML M0患者的发病年龄显著更低(中位年龄46岁对63岁,P = 0.004)。疾病定位和血液学表现存在显著差异:与AML M0相比,CD7 + CD56 + AML显示髓外受累更频繁,循环白血病原始细胞更少,贫血和血小板减少症更少。细胞遗传学异常也很独特,因为在CD7 + CD56 + M0中未发现5q异常。年龄小于46岁的CD7 + CD56 + M0患者预后较差(P = 0.03)。多变量分析显示,CD7 + CD56 +表型是AML M0的一个显著预后因素,年龄、循环原始细胞百分比和5号染色体异常也是如此。这些发现表明,AML M0由需要分别管理的异质性亚组组成,CD7 + CD56 + M0构成AML M0的一个独特亚型。