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Abnormalities on brain MR images during the course of familial haemophagocytic lymphohistocytosis in a child. A case report

Sundgren, P C LU ; Geijer, B LU ; Békássy, A N LU and Dictor, M LU (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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organization
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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
John Wiley & Sons
external identifiers
  • scopus:0033628563
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
2017-01-01 08:23:29
@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},
  keyword      = {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    = {John Wiley & Sons},
  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},
}