Abnormalities on brain MR images during the course of familial haemophagocytic lymphohistocytosis in a child. A case report
(2000) In Acta Radiologica 41(1). p.6-22- Abstract
PURPOSE: To describe and report the neuroradiological findings and clinical features in a patient with familial haemophagocytic lymphohistocytosis (FHL), a rare hereditary immune dysregulation in early childhood characterised by multisystem involvement, including in approximately 30% of cases also the central nervous system (CNS).
MATERIAL AND METHODS: Serial brain MR examinations were carried out in a 4.5-year-old boy with FHL, finally complicated with Epstein-Barr virus (EBV)-driven posttransplantation lymphoma.
RESULTS: Multiple brain MR examinations before and after contrast enhancement showed discrete perivascular non-enhancing areas of high signal intensity on T2 images, and later also an enhancing lesion in the right... (More)
PURPOSE: To describe and report the neuroradiological findings and clinical features in a patient with familial haemophagocytic lymphohistocytosis (FHL), a rare hereditary immune dysregulation in early childhood characterised by multisystem involvement, including in approximately 30% of cases also the central nervous system (CNS).
MATERIAL AND METHODS: Serial brain MR examinations were carried out in a 4.5-year-old boy with FHL, finally complicated with Epstein-Barr virus (EBV)-driven posttransplantation lymphoma.
RESULTS: Multiple brain MR examinations before and after contrast enhancement showed discrete perivascular non-enhancing areas of high signal intensity on T2 images, and later also an enhancing lesion in the right caudate nucleus.
CONCLUSION: FHL should be included in the differential diagnosis of patchy white matter abnormalities in young patients. EBV-driven post-transplantation lymphoma, which may present as meningial and/or parenchymal CNS infiltration, is a differential diagnostic problem.
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- author
- Sundgren, P C
LU
; Geijer, B LU ; Békássy, A N LU and Dictor, M LU
- organization
- publishing date
- 2000-01
- type
- Contribution to journal
- publication status
- published
- keywords
- Brain, Child, Preschool, Diagnosis, Differential, Histiocytosis, Non-Langerhans-Cell, Humans, Infant, Infant, Newborn, Magnetic Resonance Imaging, Male
- in
- Acta Radiologica
- volume
- 41
- issue
- 1
- pages
- 5 pages
- publisher
- SAGE Publications
- external identifiers
-
- scopus:0033628563
- pmid:10665865
- ISSN
- 0284-1851
- language
- English
- LU publication?
- yes
- id
- 607b070d-c940-46b0-860c-9f908743f260
- date added to LUP
- 2016-04-20 13:27:11
- date last changed
- 2024-01-04 02:30:56
@article{607b070d-c940-46b0-860c-9f908743f260, abstract = {{<p>PURPOSE: To describe and report the neuroradiological findings and clinical features in a patient with familial haemophagocytic lymphohistocytosis (FHL), a rare hereditary immune dysregulation in early childhood characterised by multisystem involvement, including in approximately 30% of cases also the central nervous system (CNS).</p><p>MATERIAL AND METHODS: Serial brain MR examinations were carried out in a 4.5-year-old boy with FHL, finally complicated with Epstein-Barr virus (EBV)-driven posttransplantation lymphoma.</p><p>RESULTS: Multiple brain MR examinations before and after contrast enhancement showed discrete perivascular non-enhancing areas of high signal intensity on T2 images, and later also an enhancing lesion in the right caudate nucleus.</p><p>CONCLUSION: FHL should be included in the differential diagnosis of patchy white matter abnormalities in young patients. EBV-driven post-transplantation lymphoma, which may present as meningial and/or parenchymal CNS infiltration, is a differential diagnostic problem.</p>}}, author = {{Sundgren, P C and Geijer, B and Békássy, A N and Dictor, M}}, issn = {{0284-1851}}, keywords = {{Brain; Child, Preschool; Diagnosis, Differential; Histiocytosis, Non-Langerhans-Cell; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male}}, language = {{eng}}, number = {{1}}, pages = {{6--22}}, publisher = {{SAGE Publications}}, series = {{Acta Radiologica}}, title = {{Abnormalities on brain MR images during the course of familial haemophagocytic lymphohistocytosis in a child. A case report}}, volume = {{41}}, year = {{2000}}, }