Limits...
CNS involvement in hemophagocytic lymphohistiocytosis: CT and MR findings.

Chung TW - Korean J Radiol (2007 Jan-Feb)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Diagnostic Radiology, Chonnam National University Hospital, 8 Hack-dong, Dong-gu, Gwangju 501-757, Korea. twchung@jnu.ac.kr

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.

Show MeSH

Related in: MedlinePlus

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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC2626691&req=5

Figure 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).

Mentions: The brain CT showed multiple irregular, thick walled, ring enhancing nodules in the both cerebral hemispheres and the right cerebellum. These lesions were scattered mainly at the white-gray matter junction, and the examination showed marked perilesional edema with a mass effect to the adjacent ventricle. Some of these lesions had small calcified foci (Figs. 1A, B). In spite of the antibiotic and antifungal therapies administered immediately after the admission because of suspected multiple brain abscesses that are typical of immunocompromised patients, the repeated postcontrast brain CT performed 14 days after the admission showed an increased size of the ring enhancing lesions. Multiple peripheral enhancing lesions surrounded by edema were noted on the brain MR imaging performed 15 days after the admission. On the T2-weighted MR image, the central portion of some lesions showed low signal intensity due to the calcifications noted on CT. The majority of the lesions showed high signal intensity on the T1-weighted MR image, and this was possibly due to internal hemorrhage. The diffusion-weighted MR image (DWI) (b = 1,000 mm2/s) revealed a decreased signal intensity change within the ring enhancing lesions (Figs. 1C-E). These findings of DWI ruled out the possible diagnosis of pyogenic or fungal abscess. The suspected diagnoses were HLH, metastasis or a toxoplasma abscess.


CNS involvement in hemophagocytic lymphohistiocytosis: CT and MR findings.

Chung TW - Korean J Radiol (2007 Jan-Feb)

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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2626691&req=5

Figure 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).
Mentions: The brain CT showed multiple irregular, thick walled, ring enhancing nodules in the both cerebral hemispheres and the right cerebellum. These lesions were scattered mainly at the white-gray matter junction, and the examination showed marked perilesional edema with a mass effect to the adjacent ventricle. Some of these lesions had small calcified foci (Figs. 1A, B). In spite of the antibiotic and antifungal therapies administered immediately after the admission because of suspected multiple brain abscesses that are typical of immunocompromised patients, the repeated postcontrast brain CT performed 14 days after the admission showed an increased size of the ring enhancing lesions. Multiple peripheral enhancing lesions surrounded by edema were noted on the brain MR imaging performed 15 days after the admission. On the T2-weighted MR image, the central portion of some lesions showed low signal intensity due to the calcifications noted on CT. The majority of the lesions showed high signal intensity on the T1-weighted MR image, and this was possibly due to internal hemorrhage. The diffusion-weighted MR image (DWI) (b = 1,000 mm2/s) revealed a decreased signal intensity change within the ring enhancing lesions (Figs. 1C-E). These findings of DWI ruled out the possible diagnosis of pyogenic or fungal abscess. The suspected diagnoses were HLH, metastasis or a toxoplasma abscess.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Diagnostic Radiology, Chonnam National University Hospital, 8 Hack-dong, Dong-gu, Gwangju 501-757, Korea. twchung@jnu.ac.kr

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.

Show MeSH
Related in: MedlinePlus