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J Neuroimmunol. 2014 Jul 15;272(1-2):67-75. doi: 10.1016/j.jneuroim.2014.04.016. Epub 2014 May 10.

Immune complex formation and in situ B-cell clonal expansion in human cerebral cavernous malformations.

Author information

1
Neurovascular Surgery Program, Section of Neurosurgery, Department of Surgery, University of Chicago Medicine and Biological Sciences, USA.
2
Section of Rheumatology, Department of Medicine and The Knapp Center for Lupus and Immunology Research, University of Chicago Medicine and Biological Sciences, USA.
3
Section of Transplantation, Department of Surgery, University of Chicago Medicine and Biological Sciences, USA.
4
Neurovascular Surgery Program, Section of Neurosurgery, Department of Surgery, University of Chicago Medicine and Biological Sciences, USA. Electronic address: iawad@uchicago.edu.

Abstract

Cerebral cavernous malformations (CCMs) represent clusters of dilated vascular channels, predisposing to hemorrhagic stroke and seizures. They are associated with defective blood brain barrier, hemorrhages of different ages and a robust inflammatory cell infiltrate. We report for the first time evidence of co-localized IgG and complement membrane attack complexes in CCM lesions. CD4(+) and CD8(+) T-cells are aggregated with CD20(+) B-cells. And IgG repertoire analyses demonstrate in situ B-cell clonal expansion and antigen-driven affinity maturation in CCMs. These results suggest an organ-intrinsic adaptive immune response in CCMs that should be further characterized as a potential therapeutic target.

KEYWORDS:

Antibody; Antigen; Cerebral cavernous malformations; Clonal expansion; IgG; Lymphocyte

PMID:
24864012
DOI:
10.1016/j.jneuroim.2014.04.016
[Indexed for MEDLINE]
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