CNS involvement in hemophagocytic lymphohistiocytosis: CT and MR findings

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder that is characterized by proliferation of benign histiocytes, and this commonly involves the liver, spleen, lymph nodes, bone marrow and central nervous system (CNS). We report here on the CT and MR imaging findings in a case of CNS HLH that showed multiple ring enhancing masses mimicking abscess or another mass on the CT and MR imaging.

Fig. 1

A 3-year-old boy with the hemophagocytic syndrome and who presented seizures and fever. A. On precontrast enhancement CT, several small parenchymal calcifications (arrowheads) can be seen in the subcortical area of both cerebral hemispheres. B. Contrast-enhanced CT reveals several thick walled peripheral enhancing lesions (arrow) with surrounding perilesional edema. Focal internal calcifications were also noted. C. The T2 weighted image reveals mixed signal intensity lesions with massive perilesional edema. The central, signal void portion of the right frontal mass shows calcification (arrowhead). D. The contrast enhanced T1 weighted image reveals irregular, thick, ring enhancement. E. No diffusion restriction in the lesions is noted on the diffusion weighted image. F. Many foamy histiocytes and atypical lymphocytes infiltrations are observed (H & E stain; original magnification, ×400).