Hemophagocytic lymphohistiocytosis in children: abdominal US findings within 1 week of presentation.
OBJECTIVE
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.


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 4(1/2) 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.


CONCLUSIONS
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.
Schmidt
Matthias H
MH
Department of Diagnostic Radiology, Dalhousie University, IWK Health Centre, 5850/5980 University Ave, Halifax, NS, Canada B3J 3G9. matthias.schmidt@iwk.nshealth.ca
Sung
Lillian
L
Shuckett
Bruce M
BM
D000005:Abdomen / Q000000981:diagnostic imaging*; D001201:Ascites / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005704:Gallbladder / Q000473:pathology; D005705:Gallbladder Diseases / Q000000981:diagnostic imaging; D006529:Hepatomegaly / Q000000981:diagnostic imaging; D015616:Histiocytosis, Non-Langerhans-Cell / Q000000981:diagnostic imaging*; D006801:Humans; D006984:Hypertrophy / Q000000981:diagnostic imaging; D007223:Infant; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000000981:diagnostic imaging; D008206:Lymphatic Diseases / Q000000981:diagnostic imaging; D008297:Male; D010996:Pleural Effusion / Q000000981:diagnostic imaging; D011169:Portal Vein / Q000000981:diagnostic imaging; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013163:Splenomegaly / Q000000981:diagnostic imaging; D014463:Ultrasonography