Department of Pediatric Oncology and Hematology, University Hospital of Caen, Avenue de La Côte de Nacre, 14000, Caen, France.
Pediatric Intensive Care Unit, CHU de Caen, 14000, Caen, France.
Ann Hematol. 2023 Mar;102(3):563-570. doi: 10.1007/s00277-022-05024-1. Epub 2022 Nov 12.
The clinical presentation of chronic myeloid leukemia (CML) at diagnosis differs in children compared to adults. At younger age, anemia appears to be frequent at diagnosis, but its prevalence and its impact on prognosis are not well known. In the International Registry of Childhood CML, we selected children and adolescents in chronic phase at diagnosis of CML and treated upfront with imatinib. We examined their hemoglobin level at diagnosis according to the WHO grades to assess the prevalence of anemia and its impact on response to tyrosine kinase inhibitors (TKIs). Data on 430 patients were included. Anemia at diagnosis was observed in 350 patients (81%), with a mean hemoglobin level of 96.4 g/l (SD 23.6). Among them, 182 patients (52%) presented with moderate anemia and 110 (31%) with severe anemia while 58 (17%) had mild anemia. Compared with mild and no anemia, moderate and severe forms were significantly associated with younger age at diagnosis, asthenia, splenomegaly, and increased leukocyte and basophil counts. Delays in achieving major and deep molecular responses were significantly increased for patients with moderate and severe anemia, and also failure of imatinib treatment was more frequent in these two sub-cohorts. However, hemoglobin level was not significantly associated with survival. Anemia at diagnosis of pediatric CML was frequent and may be considered as a prognostic factor.
慢性髓性白血病(CML)在诊断时的临床表现在儿童与成人之间存在差异。在较年轻的年龄,贫血似乎在诊断时很常见,但它的患病率及其对预后的影响尚不清楚。在儿童 CML 国际注册处,我们选择了在诊断时患有 CML 的慢性期的儿童和青少年,并接受伊马替尼的一线治疗。我们根据世界卫生组织(WHO)的分级检查了他们在诊断时的血红蛋白水平,以评估贫血的患病率及其对酪氨酸激酶抑制剂(TKI)反应的影响。纳入了 430 例患者的数据。在 350 例患者(81%)中观察到诊断时的贫血,平均血红蛋白水平为 96.4 g/l(SD 23.6)。其中,182 例患者(52%)为中度贫血,110 例患者(31%)为重度贫血,58 例患者(17%)为轻度贫血。与轻度和无贫血相比,中度和重度贫血与诊断时年龄较小、乏力、脾肿大以及白细胞和嗜碱性粒细胞计数增加显著相关。对于中度和重度贫血的患者,达到主要和深度分子反应的延迟明显增加,而且这两个亚组中伊马替尼治疗失败的频率也更高。然而,血红蛋白水平与生存无显著相关性。儿科 CML 诊断时的贫血很常见,可被视为一个预后因素。