Schmidt Matthias H, Sung Lillian, Shuckett Bruce M
Department of Diagnostic Radiology, Dalhousie University, IWK Health Centre, 5850/5980 University Ave, Halifax, NS, Canada B3J 3G9.
Radiology. 2004 Mar;230(3):685-9. doi: 10.1148/radiol.2303030223.
To evaluate the ultrasonographic (US) findings at presentation in a series of children who received a diagnosis of hemophagocytic lymphohistiocytosis (HLH) at a single institution.
All available results of imaging studies of children who received a diagnosis of HLH between January 1985 and June 2000 were retrieved. For nine patients, abdominal US images obtained within 1 week of presentation to the hospital were reviewed retrospectively by two radiologists who were aware of the diagnosis and of the original interpretation of each study. US images were examined for evidence of splenomegaly, hepatomegaly, ascites, gallbladder wall thickening, increased periportal echogenicity, lymphadenopathy, pleural effusion, and nephromegaly. Any other abnormalities were also recorded. Differences in interpretation were resolved by consensus. The patients ranged in age from 2 months to 4(1/2) years. The male-to-female ratio was 5:4.
Findings at presentation included splenomegaly (in eight of the nine children), hepatomegaly (in seven children), ascites (in six children), gallbladder wall thickening (in six children), increased periportal echogenicity (in three children), lymphadenopathy (in three children), and pleural effusion (in two children). Miscellaneous findings in individual patients included coarse hepatic echotexture with a single 9-mm hypoechoic focus in the liver, multiple hypoechoic foci in the spleen, nephromegaly, gallstone, increased renal cortical echogenicity, and mural thickening of the duodenum.
In the appropriate clinical setting, the differential diagnosis of a combination of hepatosplenomegaly, ascites, gallbladder wall thickening, increased periportal echogenicity, lymphadenopathy, and/or pleural effusion should include HLH.
评估在一家机构被诊断为噬血细胞性淋巴组织细胞增生症（HLH）的一系列儿童患者就诊时的超声（US）检查结果。
检索1985年1月至2000年6月期间被诊断为HLH的儿童患者所有可用的影像学研究结果。对于9例患者，由两名知晓诊断及每项研究原始解读的放射科医生对入院1周内获得的腹部US图像进行回顾性分析。检查US图像以寻找脾肿大、肝肿大、腹水、胆囊壁增厚、门静脉周围回声增强、淋巴结病、胸腔积液和肾肿大的证据。还记录任何其他异常情况。解读差异通过达成共识解决。患者年龄范围为2个月至4.5岁。男女比例为5:4。
就诊时的检查结果包括脾肿大（9例儿童中的8例）、肝肿大（7例儿童）、腹水（6例儿童）、胆囊壁增厚（6例儿童）、门静脉周围回声增强（3例儿童）、淋巴结病（3例儿童）和胸腔积液（2例儿童）。个别患者的其他杂项检查结果包括肝脏回声粗糙伴肝脏内一个9毫米的低回声灶、脾脏内多个低回声灶、肾肿大、胆结石、肾皮质回声增强以及十二指肠壁增厚。
在适当的临床情况下，肝脾肿大、腹水、胆囊壁增厚、门静脉周围回声增强、淋巴结病和/或胸腔积液合并出现时的鉴别诊断应包括HLH。
