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DOI: 10.1148/radiol.2303030223
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(Radiology 2004;230:685-689.)
© RSNA, 2004

Pediatric Imaging

Hemophagocytic Lymphohistiocytosis in Children: Abdominal US Findings within 1 Week of Presentation1

Matthias H. Schmidt, MD, Lillian Sung, MD and Bruce M. Shuckett, MD

1 From the Department of Diagnostic Radiology, Dalhousie University and Department of Diagnostic Imaging, IWK Health Centre, 5850/5980 University Ave, Halifax, NS, Canada B3J 3G9 (M.H.S.); Departments of Pediatrics and Health Policy Management & Evaluation, University of Toronto and Division of Hematology and Oncology, Hospital for Sick Children, Toronto, Ontario, Canada (L.S.); and Department of Diagnostic Imaging, Scarborough Hospital, General Division, Scarborough, Ontario, Canada (B.M.S.). From the 2002 RSNA scientific assembly. Received February 7, 2003; revision requested April 25; revision received June 9; accepted July 23. Address correspondence to M.H.S. (e-mail: matthias.schmidt@iwk.nshealth.ca).

PURPOSE: 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.

MATERIALS AND METHODS: 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 41/2 years. The male-to-female ratio was 5:4.

RESULTS: 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.

CONCLUSION: 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.

© RSNA, 2004

Index terms: Abdomen, US, 70.1298 • Blood, diseases, 70.659 • Ultrasound (US), in infants and children