İrken Gülersu, Ören Hale, Öniz Haldun, Çetingül Nazan, Vergin Canan, Atabay Berna, Gülen Hüseyin, Türker Meral, Kantar Mehmet, Yılmaz Şebnem
Turk J Haematol. 2006 Sep 5;23(3):142-6.
Hyperleukocytosis, defined as a peripheral leukocyte count ≥ 100x109/L, is seen in 5-20% of newly diagnosed cases of childhood leukemia and is a poor prognostic factor. In this study, we aimed to examine the presenting clinical and laboratory features, complications, and treatment outcome of 47 children with acute lymphoblastic leukemia (ALL) and hyperleukocytosis who were diagnosed and treated in four medical centers of İzmir between January 1990 and January 2001. The median age was 5.0 years (range: 0.1-16.3 years). Median white blood cell count was 495x109/L (range: 107x109/L- 794x109/L). Forty-two of 47 patients (90%) had hepatosplenomegaly, 5 (11%) had respiratory distress, 3 (6%) had neurologic symptoms, 3 (6%) had diffuse cervical lymphadenopathy, and 3 (6%) had acute renal failure at admission. Ten of 47 patients (21%) had central nervous system involvement, and 17 (36%) had mediastinal mass. Ten patients (21%) had coagulopathy and 15 patients (32%) had metabolic complications (8 patients had hyperuricemia, 4 had hyperphosphatemia, 2 had hyperuricemia, hyperphosphatemia and hypercalcemia, and 1 had hypocalcemia) before the initiation of therapy. Forty of 47 patients (85%) with hyperleukocytosis were effectively managed with intravenous hydration, alkalinization, and allopurinol therapy. Early death during remission induction therapy occurred in 5 patients (11%) with respiratory distress and sepsis. Kaplan-Meier estimates of event free survival and overall survival were 37.0% and 40.5%, respectively.
高白细胞血症定义为外周血白细胞计数≥100×10⁹/L,在5% - 20%的儿童白血病新诊断病例中可见,是一个不良预后因素。在本研究中,我们旨在检查1990年1月至2001年1月期间在伊兹密尔的四个医疗中心诊断和治疗的47例急性淋巴细胞白血病(ALL)合并高白细胞血症患儿的临床表现、实验室检查特征、并发症及治疗结果。中位年龄为5.0岁(范围:0.1 - 16.3岁)。中位白细胞计数为495×10⁹/L(范围:107×10⁹/L - 794×10⁹/L)。47例患者中有42例(90%)有肝脾肿大,5例(11%)有呼吸窘迫,3例(6%)有神经症状,3例(6%)有弥漫性颈部淋巴结肿大,3例(6%)入院时出现急性肾衰竭。47例患者中有10例(21%)有中枢神经系统受累,17例(36%)有纵隔肿块。10例患者(21%)有凝血功能障碍,15例患者(32%)在开始治疗前有代谢并发症(8例有高尿酸血症,4例有高磷血症,2例有高尿酸血症、高磷血症和高钙血症,1例有低钙血症)。47例高白细胞血症患者中有40例(85%)通过静脉补液、碱化及别嘌醇治疗得到有效控制。诱导缓解治疗期间,5例(11%)有呼吸窘迫和败血症的患者早期死亡。无事件生存率和总生存率的Kaplan - Meier估计分别为37.0%和40.5%。