Chateil J, Brun M, Perel Y, Pillet P, Micheau M, Diard F
Service de Radiologie A, Hôpital Pellegrin, Place A. Raba Léon, F-33076 Bordeaux Cedex, France.
Eur Radiol. 1999;9(3):474-7. doi: 10.1007/s003300050695.
Hemophagocytic lymphohistiocytosis, a variant of histiocytosis, is characterized by an uncontrolled activation of the cellular immune system, including hepatic mononuclear phagocytic cells. Abdominal ultrasound findings in children are evaluated in this disease. We present six pediatric cases, two with familial and four with sporadic hemophagocytic lymphohistiocytosis, examined by abdominal sonography. Three signs were frequently observed: thickening of the gallbladder wall (all cases), increased periportal echogenicity (four cases), and enlarged lymph nodes in the porta hepatis (four cases). Hepatomegaly, splenomegaly, and ascitic fluid may also be found. These imaging findings are not specific and may be seen in viral hepatitis. However, once hepatitis is excluded, they may suggest the diagnosis of hemophagocytic lymphohistiocytosis in a critically ill child. A bone smear must be done to establish the diagnosis.
噬血细胞性淋巴组织细胞增生症是组织细胞增多症的一种变体，其特征是包括肝单核吞噬细胞在内的细胞免疫系统不受控制地激活。本文对患有该疾病的儿童腹部超声检查结果进行评估。我们呈现6例儿科病例，其中2例为家族性噬血细胞性淋巴组织细胞增生症，4例为散发性噬血细胞性淋巴组织细胞增生症，均接受了腹部超声检查。经常观察到三个征象：胆囊壁增厚（所有病例）、门静脉周围回声增强（4例）和肝门淋巴结肿大（4例）。也可能发现肝肿大、脾肿大和腹水。这些影像学表现不具有特异性，在病毒性肝炎中也可见。然而，一旦排除肝炎，这些表现可能提示重症患儿患有噬血细胞性淋巴组织细胞增生症。必须进行骨髓涂片以明确诊断。
