Department of Paediatrics and Adolescent Medicine, The University Hospital Rigshospitalet, Copenhagen, Denmark.
Pediatr Blood Cancer. 2014 Aug;61(8):1416-21. doi: 10.1002/pbc.24981. Epub 2014 Feb 12.
Central nervous system (CNS) involvement in childhood acute lymphoblastic leukemia (ALL) remains a therapeutic challenge.
To explore leukemia characteristics of patients with CNS involvement at ALL diagnosis, we analyzed clinical features and early treatment response of 744 patients on Nordic-Baltic trials. CNS status was classified as CNS1 (no CSF blasts), CNS2 (<5 leukocytes/µl CSF with blasts), CNS3 (≥5 leukocytes/µl with blasts or signs of CNS involvement), TLP+ (traumatic lumbar puncture with blasts), and TLP- (TLP with no blasts).
Patients with CNS involvement had higher leukocyte count compared with patients with CNS1 (P < 0.002). Patients with CNS3 more often had T-ALL (P < 0.001) and t(9;22)(q34;q11)[BCR-ABL1] (P < 0.004) compared with patients with CNS1. Among patients with CNS involvement headache (17%) and vomiting (14%) were most common symptoms. Symptoms or clinical findings were present among 27 of 54 patients with CNS3 versus only 7 of 39 patients with CNS2 and 15 of 75 patients with TLP+ (P < 0.001). The majority of patients with CNS involvement received additional induction therapy. The post induction bone marrow residual disease level did not differ between patients with CNS involvement and patients with CNS1 (P > 0.15). The 12-year event-free survival for patients with leukemic mass on neuroimaging did not differ from patients with negative or no scan (0.50 vs. 0.60; P = 0.7) or between patients with symptoms or signs suggestive of CNS leukemia and patients without such characteristics (0.50 vs. 0.61; P = 0.2).
CNS involvement at diagnosis is associated with adverse prognostic features but does not indicate a less chemosensitive leukemia.
儿童急性淋巴细胞白血病（ALL）的中枢神经系统（CNS）受累仍然是一个治疗挑战。
为了探讨 ALL 诊断时中枢神经系统受累患者的白血病特征，我们分析了北欧-波罗的海试验中的 744 名患者的临床特征和早期治疗反应。CNS 状态分为 CNS1（无 CSF blasts）、CNS2（CSF 中有 <5 个白细胞/µl 但有blasts）、CNS3（≥5 个白细胞/µl 并有blasts 或 CNS 受累迹象）、TLP+（有blasts 的创伤性腰椎穿刺）和 TLP-（无blasts 的 TLP）。
与 CNS1 患者相比，CNS 受累患者的白细胞计数更高（P<0.002）。与 CNS1 患者相比，CNS3 患者更常患有 T-ALL（P<0.001）和 t(9;22)(q34;q11)[BCR-ABL1]（P<0.004）。在 CNS 受累的患者中，头痛（17%）和呕吐（14%）是最常见的症状。在 54 例 CNS3 患者中有 27 例存在症状或临床体征，而在 39 例 CNS2 患者中仅有 7 例，在 75 例 TLP+患者中仅有 15 例（P<0.001）。大多数 CNS 受累患者接受了额外的诱导治疗。诱导后骨髓残留疾病水平在 CNS 受累患者与 CNS1 患者之间没有差异（P>0.15）。神经影像学有白血病肿块的患者的 12 年无事件生存与阴性或无扫描患者无差异（0.50 与 0.60；P=0.7），或与有 CNS 白血病症状或体征的患者与无这些特征的患者无差异（0.50 与 0.61；P=0.2）。
诊断时的 CNS 受累与不良预后特征相关，但并不表明白血病对化疗的敏感性较低。
