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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2023; 33(04): 548-554
DOI: 10.1055/s-0043-1772495
Case Series

Imaging Spectrum of Neurological Manifestations of Hemophagocytic Lymphohistiocytosis in Pediatrics: A Case Series

Geethapriya Sivaramalingam
1   Department of Radiology, Apollo Speciality Hospital, Chennai, Tamil Nadu, India
,
Afiya VK 
1   Department of Radiology, Apollo Speciality Hospital, Chennai, Tamil Nadu, India
,
Bagyam Raghavan 
1   Department of Radiology, Apollo Speciality Hospital, Chennai, Tamil Nadu, India
,
Jayaraj Govindaraj 
1   Department of Radiology, Apollo Speciality Hospital, Chennai, Tamil Nadu, India
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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon condition, which can result either from a primary genetic abnormality affecting children or secondary to various conditions like malignancy or infection predominantly in adults. HLH is associated with immune dysregulation, resulting in an uncontrolled overproduction and infiltration of lymphocytes and histiocytes. The infiltration predominantly involves liver, spleen, lymph nodes, and central nervous system. Neuroimaging features on magnetic resonance imaging are highly nonspecific and variable. The most typical findings include periventricular white matter hyperintensities and diffuse atrophy. Ring or nodular enhancing or nonenhancing focal parenchymal lesions may be seen. Here, we present three pediatric cases of primary HLH with a wide spectrum of imaging findings involving cerebral and cerebellar cortex, white matter, deep gray matter, and brain stem. The findings in these patients range from small nonenhancing hemorrhagic lesions and enhancing small lesions to ill-defined mass with mass effect and midline shift. Lesions in deep gray matter including thalamus, basal ganglia, and also brain stem in HLH are rarely described in literature. Early diagnosis of HLH and timely management can improve the course of the disease.

Keywords

hemophagocytic lymphohistiocytosis - neuroimaging - pediatric


Publication History

Article published online:
21 August 2023

© 2023. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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