Imashuku S, Hlbi S, Todo S
Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan.
J Pediatr. 1997 Mar;130(3):352-7. doi: 10.1016/s0022-3476(97)70195-1.
The current status of hemophagocytic lymphohistiocytosis (HLH) in infants and children has been studied.
Eighty-two cases of pediatric HLH, for which there was no confirmed familial inheritance, were comparatively studied between 36 patients less than 2 years of age and 46 patients more than 2 years of age.
In all cases, persistent fever, cytopenia, liver dysfunction, and hepatosplenomegaly were the most frequently noted symptoms. Hyperferritinemia (> 1000 micrograms/L) and elevated blood levels of lactate dehydrogenase (> 1000 IU/L) were observed in 90% and 89.7%, respectively. These figures were considerably higher than for either hypertriglyceridemia (> 2 mmol/L) (50%) or hypofibrinogenemia (< 1.5 gm/L) (57.4%), indicating that increased serum ferritin and lactate dehydrogenase concentrations might be good diagnostic parameters for HLH. These parameters are nonspecific but are of follow-up and prognostic value in these HLH cases. No differences were found in clinical signs and symptoms or other laboratory findings for the two age groups. Immunochemotherapy was administered in the similar regimens to patients in both groups. Of the 82 patients, 13 (15.9%) succumbed to a fatal course within 2 months after diagnosis, and Kaplan-Meir analysis for all cases predicted the overall disease-free patient survival at 4 years from the onset of disease to be 57.2% (95% confidence interval (CI), 45.1% to 69.3%). There was a slightly, but not statistically significant, poorer prognosis for the younger patients: 44.2% (95% CI, 26.0% to 62.4%) survival for the infant group versus 67.2% (95% CI, 51.8% to 82.6%) survival for the older group (p = 0.0569).
Refinement of the treatment is mandatory to improve the outcome of HLH in both infants and older pediatric patients.
研究婴儿和儿童噬血细胞性淋巴组织细胞增生症（HLH）的现状。
对82例无确诊家族遗传的儿童HLH病例进行比较研究，其中36例年龄小于2岁，46例年龄大于2岁。
所有病例中，持续发热、血细胞减少、肝功能障碍和肝脾肿大是最常见的症状。分别有90%和89.7%的患者出现高铁蛋白血症（>1000微克/升）和血乳酸脱氢酶水平升高（>1000国际单位/升）。这些数字显著高于高甘油三酯血症（>2毫摩尔/升）（50%）或低纤维蛋白原血症（<1.5克/升）（57.4%），表明血清铁蛋白和乳酸脱氢酶浓度升高可能是HLH的良好诊断参数。这些参数是非特异性的，但对这些HLH病例具有随访和预后价值。两个年龄组在临床体征、症状或其他实验室检查结果方面未发现差异。两组患者均采用相似的免疫化疗方案。82例患者中，13例（15.9%）在诊断后2个月内死于致命病程，对所有病例的Kaplan-Meir分析预测，从疾病发作起4年时无病患者的总体生存率为57.2%（95%置信区间（CI），45.1%至69.3%）。较年轻患者的预后略差，但无统计学意义：婴儿组生存率为44.2%（95%CI，26.0%至62.4%），而较大儿童组生存率为67.2%（95%CI，51.8%至82.6%）（p = 0.0569）。
必须改进治疗方法以改善婴儿和大龄儿科患者HLH的治疗结果。
