Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 30, 8036, Graz, Austria.
Support Care Cancer. 2013 Dec;21(12):3519-23. doi: 10.1007/s00520-013-1937-x. Epub 2013 Aug 24.
Intensification of antileukemic treatment and progress in supportive management have improved the survival rates of children with acute myeloid leukemia (AML). However, morbidity and early mortality in these patients are still very high, especially in children with acute monoblastic leukemia (AML FAB M5). Inflammatory syndromes complicating the management of these children after application of cytosine arabinoside and due to hyperleukocytosis at initial presentation have been reported. Hemophagocytic lymphohistiocytosis (HLH) has been described as a serious and life-threatening acute complication during treatment of different oncologic entities; however, data on HLH in children with AML FAB M5 are extremely rare.
A retrospective study of all children with AML FAB M5 treated at our institution between 1993 and 2013 was performed to describe the clinical characteristics of patients who developed an inflammatory syndrome with HLH during oncologic treatment.
Three of 10 children developed an inflammatory syndrome with fever, elevation of C-reactive protein, hyperferritinemia, elevation of soluble interleukin-2, and hemophagocytosis during prolonged aplasia following the first cycle of chemotherapy not responding to broad-spectrum antibiotics. No infectious agents could be identified; the initial symptoms occurred 17, 18, and 28 days after diagnosis of AML, respectively. The children immediately responded to dexamethasone; however, the same syndrome was observed again after the second cycle of chemotherapy and, in one patient, also after the third cycle.
Treating physicians should be aware of an inflammatory syndrome resembling HLH in children with monoblastic leukemia since this problem might extremely complicate management and supportive care of these children. The co-incidence of monoblastic leukemia with HLH might be explained by cytokines released from the monoblastic leukemic cells themselves.
强化抗白血病治疗和支持性治疗管理的进步提高了儿童急性髓细胞白血病（AML）的生存率。然而，这些患者的发病率和早期死亡率仍然非常高，尤其是患有急性单核细胞白血病（AML FAB M5）的儿童。在应用阿糖胞苷后，由于初始高白细胞血症，这些儿童的管理中出现了炎症综合征，已有报道。噬血细胞性淋巴组织细胞增生症（HLH）已被描述为在不同肿瘤实体治疗过程中一种严重且危及生命的急性并发症；然而，AML FAB M5 患儿中 HLH 的数据极为罕见。
对我院 1993 年至 2013 年间治疗的所有 AML FAB M5 患儿进行回顾性研究，以描述在肿瘤治疗期间发生炎症综合征伴 HLH 的患者的临床特征。
10 例患儿中有 3 例在第一周期化疗后长时间骨髓抑制期间出现发热、C 反应蛋白升高、高铁蛋白血症、可溶性白细胞介素-2 升高和噬血细胞现象的炎症综合征，广谱抗生素治疗无效。未发现感染性病原体；初始症状分别出现在 AML 诊断后 17、18 和 28 天。患儿立即对地塞米松有反应；然而，在第二周期化疗后再次观察到相同的综合征，在 1 例患儿中，在第三周期化疗后也观察到相同的综合征。
治疗医生应注意单核细胞白血病患儿出现类似于 HLH 的炎症综合征，因为这可能会极大地复杂化这些患儿的管理和支持性护理。单核细胞白血病伴 HLH 的巧合可能是由单核白血病细胞本身释放的细胞因子引起的。
