PMID- 34284548
OWN - NLM
STAT- MEDLINE
DCOM- 20220113
LR  - 20220113
IS  - 1509-572X (Electronic)
IS  - 1509-572X (Linking)
VI  - 59
IP  - 2
DP  - 2021
TI  - Brain aggregoma with clonal B-cell perivascular proliferation detected by 
      next-generation sequencing. A case report and review of the literature.
PG  - 205-211
LID - 44156 [pii]
LID - 10.5114/fn.2021.106405 [doi]
AB  - Light-chain deposition disease (LCDD), a rare type of monoclonal immunoglobulin 
      deposition disease, can be presented as systemic or localized, very rarely 
      affecting central nervous system (CNS). Only 10 cases of CNS-LCDD have been 
      described so far. We present an eleventh case of cerebral tumour-like LCDD, 
      called aggregoma, and compare it with previously reported cases. A 49-year-old 
      patient was admitted to the hospital due to a first generalized epileptic 
      seizure. Magnetic resonance imaging (MRI) showed focal lesion in the right 
      occipital lobe. Abundant parenchymal aggregates of pale eosinophilic material 
      were observed, Congo red negative, Thioflavin T moderately positive, and l-light 
      chain positive, but k negative in immunofluorescence with mild perivascular 
      lymphoplasmacytic infiltrates in the intervening brain tissue. Clonality testing 
      by next-generation sequencing showed the monoclonal nature of B-lymphocytes. 
      Electron microscopy showed a finely granular ultrastructure of the aggregates 
      without deposition in the vessel walls. A whole-body workup did not show any 
      extra-cerebral immune dyscrasias.
FAU - Matjasic, Alenka
AU  - Matjasic A
AD  - Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, 
      Slovenia.
FAU - Wechtersbach, Karmen
AU  - Wechtersbach K
AD  - Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, 
      Slovenia.
FAU - Kavalar, Rajko
AU  - Kavalar R
AD  - Department of Pathology, University Medical Centre Maribor, Maribor, Slovenia.
FAU - Vorsic, Matjaz
AU  - Vorsic M
AD  - Department of Neurosurgery, University Medical Centre Maribor, Maribor, Slovenia.
FAU - Mlakar, Jernej
AU  - Mlakar J
AD  - Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, 
      Slovenia.
FAU - Ravnik, Janez
AU  - Ravnik J
AD  - Department of Neurosurgery, University Medical Centre Maribor, Maribor, Slovenia.
FAU - Popovic, Mara
AU  - Popovic M
AD  - Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, 
      Slovenia.
LA  - eng
PT  - Case Reports
PT  - Review
PL  - Poland
TA  - Folia Neuropathol
JT  - Folia neuropathologica
JID - 9437431
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - *Brain/metabolism
MH  - Cell Proliferation
MH  - High-Throughput Nucleotide Sequencing
MH  - Humans
MH  - *Immunoglobulin Light Chains/metabolism
MH  - Magnetic Resonance Imaging
MH  - Middle Aged
OTO - NOTNLM
OT  - CNS-LCDD
OT  - immunoglobulin light chains
OT  - next-generation sequencing
OT  - paraproteinaemia.
OT  - aggregoma
EDAT- 2021/07/22 06:00
MHDA- 2022/01/14 06:00
CRDT- 2021/07/21 03:26
PHST- 2021/07/21 03:26 [entrez]
PHST- 2021/07/22 06:00 [pubmed]
PHST- 2022/01/14 06:00 [medline]
AID - 44156 [pii]
AID - 10.5114/fn.2021.106405 [doi]
PST - ppublish
SO  - Folia Neuropathol. 2021;59(2):205-211. doi: 10.5114/fn.2021.106405.