儿童脓毒症合并噬血细胞综合征诊断学特征分析

Diagnostic characteristics of sepsis complicated with hemophagocytic syndrome in children

目的探讨儿童脓毒症合并噬血细胞综合征的诊断学特征。方法回顾性分析2016年1月至2020年7月广东省妇幼保健院儿科重症监护室收治并确诊的37例脓毒症合并噬血细胞综合征患儿的临床症状及实验室检测结果。结果37例脓毒症合并噬血细胞综合征患儿最常见的临床症状为发热(100.00%),峰值38℃以上,其余主要特征包括肝大(32.43%)、皮疹(32.43%)、浅表淋巴结肿大(27.03%)和三凹征(21.62%)。实验室检查中,37例患儿均出现了不同程度的丙氨酸转氨酶、D-二聚体及血清铁蛋白水平升高,血红蛋白水平及血小板计数降低29例(78.38%),B淋巴细胞比例升高23例(62.16%),NK细胞比例降低24例(64.86%),天冬氨酸转氨酶升高34例(91.89%),骨髓中均发现噬血细胞。29例(78.38%)患儿病原体检测阳性;EB病毒检出率最高(48.65%);真菌以白念珠菌为主(18.92%)。单一原发感染部位以呼吸道感染为主(37.84%)。经治疗,治愈出院15例,转院治疗8例,放弃治疗12例,死亡2例。结论脓毒症合并噬血细胞综合征患儿的临床表现复杂多样,实验室及病原体检测中易出现高铁血红蛋白血症和(或)EB病毒感染,早期诊断对救治患儿的生命有重大作用。

Objective To explore the diagnostic characteristics of children with hemophagocytic lymphohistiocytosis(HLH)complicated with sepsis.MethodsBetween January 2016 and July 2020,clinical symptoms and laboratory test results of 37 children with HLH secondary to sepsis from the Pediatric Intensive Care Unit(PICU)of Guangdong Women and Children Hospital were collected retrospectively.ResultsFever(100.00%)was the most common clinical symptom in 37 children with sepsis complicated with HLH,the peak fever was over 38℃followed by hepatomegalemia(32.43%),rash(32.43%),superficial lymphadenopathy(27.03%)and trisophysis(21.62%).All 37 children showed varying degrees of high levels of alanine aminotransferase,D-dimer,and serum ferritin in laboratory tests.The hemoglobin level and platelet count decreased in 29 cases(78.38%),the proportion of B lymphocytes increased in 23 cases(62.16%),the proportion of NK cells decreased in 24 cases(64.86%),and the aspartate aminotransferase increased in 34 cases(91.89%).In the bone marrow,hemophagocytic cells were discovered.Pathogen detection was positive in 29 cases(78.38%),with EB virus having the highest detection rate(48.65%),and candida albicans being the main fungus(18.92%).The respiratory tract was the most common site of infection(37.84%).After the treatment,15 patients recovered,8 were transferred to another hospital,12 discontinued their treatment,and 2 died.ConclusionsChildren with sepsis complicated by HLH have a wide range of clinical manifestations.Methemoglobinemia and/or EB virus infection are common in laboratory and pathogen detection.Early diagnosis plays an important role in saving the lives of children.