Fitzgerald Nancy E, MacClain Kenneth L
E B Singleton Department of Diagnostic Imaging, Texas Children's Hospital, 6621 Fannin Street, MC 2-2521, TX 77030, Houston, US.,
Pediatr Radiol. 2003 Jun;33(6):392-401. doi: 10.1007/s00247-003-0894-9. Epub 2003 Mar 21.
Hemophagocytic lymphohistiocytosis (HLH) is a nonmalignant disorder of immune regulation, with overproduction of cytokines and diminished immune surveillance. Symptoms are nonspecific and may affect multiple organs, including the central nervous system. Neuroimaging findings have been described in case reports and small series; body imaging findings have not been described extensively. OBJECTIVE. To summarize findings of the most frequently performed imaging studies of the brain, chest and abdomen in patients with HLH.
Retrospective review of chest radiographs and CT, abdominal ultrasound and CT, brain CT and MRI, skeletal surveys, and autopsy data.
Twenty-five patients were diagnosed and treated for HLH at our institution over an 11-year period; 15 patients (60%) died. Common chest radiograph findings included alveolar-interstitial opacities with pleural effusions, often with rapid evolution and resolution. Hepatosplenomegaly, gallbladder wall thickening, hyperechoic kidneys and ascites were common abdominal findings, which resolved after therapy in some cases. Brain-imaging studies revealed nonspecific periventricular white-matter abnormalities, brain-volume loss and enlargement of extra-axial fluid spaces. Three infant cases, one with intracranial hemorrhage, one with multiple pathologic rib fractures and one with diaphyseal periosteal reaction involving multiple long bones on skeletal survey, raised suspicion of child abuse at presentation. Abuse was not substantiated in any case.
Clinicians and radiologists should be aware of the radiographic manifestations of HLH, which are nonspecific and overlap with infectious, inflammatory and neoplastic disorders. Findings in the chest (similar to acute respiratory distress syndrome) and abdomen may progress rapidly and then regress with institution of appropriate anti-HLH therapy. CNS findings may be progressive. In some infants, initial imaging findings may mimic nonaccidental trauma.
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫调节的非恶性疾病，伴有细胞因子过度产生和免疫监视功能减弱。症状不具特异性，可累及多个器官，包括中枢神经系统。病例报告和小样本系列研究中已描述了神经影像学表现；全身影像学表现尚未得到广泛描述。目的：总结HLH患者脑部、胸部和腹部最常用影像学检查的结果。
回顾性分析胸部X线片和CT、腹部超声和CT、脑部CT和MRI、骨骼检查及尸检数据。
在11年期间，我们机构共诊断并治疗了25例HLH患者；15例（60%）死亡。胸部X线片常见表现包括肺泡间质模糊影伴胸腔积液，通常迅速进展并消退。肝脾肿大、胆囊壁增厚、肾回声增强和腹水是常见的腹部表现，部分病例经治疗后可缓解。脑部影像学检查显示脑室周围白质非特异性异常、脑容量减少和脑外液间隙增宽。3例婴儿病例，1例有颅内出血，1例有多发性病理性肋骨骨折，1例骨骼检查显示多根长骨干骺端骨膜反应，初诊时怀疑有虐待儿童情况。但所有病例均未得到虐待的确证。
临床医生和放射科医生应了解HLH的影像学表现，这些表现不具特异性，与感染性、炎症性和肿瘤性疾病表现重叠。胸部表现（类似于急性呼吸窘迫综合征）和腹部表现可能迅速进展，随后在进行适当的抗HLH治疗后消退。中枢神经系统表现可能呈进行性。在一些婴儿中，最初的影像学表现可能类似非意外创伤。
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