Crist W, Pullen J, Boyett J, Falletta J, van Eys J, Borowitz M, Jackson J, Dowell B, Frankel L, Quddus F
Blood. 1986 Jan;67(1):135-40.
Analysis of remission induction rates for 1,117 children 18 months to 10 years of age (group 1) and 90 infants less than 18 months of age (group 2) with acute lymphoid leukemia (ALL) and of duration of continuous complete remission (CCR) for 454 in group 1 and 33 in group 2 revealed that infants fared significantly worse in both measures of outcome (P = .03 and P less than .0001). To examine potential reasons for the poor prognosis of affected infants, clinical and biologic features of their ALL were compared. Infants had higher WBC counts (P less than .001), a higher incidence of massive splenomegaly (P less than .001), massive hepatomegaly (P less than .001), more central nervous system (CNS) disease at diagnosis (P less than .01), and lower platelet counts (P less than .001). Also, their blasts were less often PAS+ (P = .02). The incidence of non(T, B, pre-B), T and pre-B immunophenotypes of ALL did not differ significantly between the two groups. However, in patients with non(T, B, pre-B) ALL, the majority (51%) of infants had common ALL antigen (CALLA)-negative blasts, as compared with only 7% in group 1 (P less than .001). Furthermore, infants with non(T, B, pre-B) cell ALL who were less than 12 months of age were almost always CALLA- (18 of 21). The blasts of children from both groups usually expressed Ia-like antigens. These data illustrate that infants with ALL have extensive and bulky disease more often than do older children and are more often affected with a prognostically unfavorable phenotype of acute leukemia (AL) which expresses Ia-like antigens but is more often PAS- and CALLA-. We believe that these clinical and biological differences predict and explain in part the observed poor response to treatment of infants with ALL.
对1117名18个月至10岁的儿童(第1组)和90名18个月以下的婴儿(第2组)急性淋巴细胞白血病(ALL)的缓解诱导率以及第1组中454名和第2组中33名儿童的持续完全缓解(CCR)持续时间进行分析后发现,婴儿在这两项预后指标上的表现均明显较差(P = 0.03,P小于0.0001)。为了探究患病婴儿预后不良的潜在原因,对他们ALL的临床和生物学特征进行了比较。婴儿的白细胞计数更高(P小于0.001),巨脾肿大(P小于0.001)、巨肝肿大(P小于0.001)的发生率更高,诊断时中枢神经系统(CNS)疾病更多(P小于0.01),血小板计数更低(P小于0.001)。此外,他们的原始细胞PAS阳性的情况较少(P = 0.02)。ALL的非(T、B、前B)、T和前B免疫表型的发生率在两组之间没有显著差异。然而,在患有非(T、B、前B)ALL的患者中,大多数(51%)婴儿的原始细胞为普通ALL抗原(CALLA)阴性,而第1组中只有7%(P小于0.001)。此外,年龄小于12个月的非(T、B、前B)细胞ALL婴儿几乎总是CALLA阴性(21例中有18例)。两组儿童的原始细胞通常都表达Ia样抗原。这些数据表明,与大龄儿童相比,ALL婴儿更常出现广泛且体积较大的疾病,并且更常受到预后不良的急性白血病(AL)表型的影响,该表型表达Ia样抗原,但更常为PAS阴性和CALLA阴性。我们认为,这些临床和生物学差异部分预测并解释了所观察到的ALL婴儿对治疗反应不佳的情况。