Relationship between autoimmunity and immunodeficiency in CLL

F. Caligaris-Cappio

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

B-CLL patients have a high prevalence of autoimmune phenomena due to polyclonal autoantibodies (Abs) restricted to blood cell self-antigens (Ag) and progressively develop a severe hypogammaglobulinemia. Autoimmunity and immunodeficiency have been related to B-CLL biological properties and cellular origin. Three major issues have emerged: 1) the relevance of B cell receptor abnormalities. More specifically, the importance of the defective expression of CD79b which may explain the faint to virtually undetectable amounts of monoclonal sig, the anomalous signal transduction and the failure to present antigens (Ag). 2) the possibility that the relentless accumulation of B-CLL cells is also under microenvironmental influences. These are brought about by the interplay of cytokines produced through cell/cell contacts among malignant B cells, macrophages and T cells. 3) the view that the very properties of accumulating anergic self-reactive CD5+ B lymphocytes may provide a bridge between autoimmunity and immune incompetence. CD5+ malignant B cells influence the cytokine production by T cells, but are inefficient Ag presenting cells (APC). As such they are a hurdle to the production of normal Ab. However, by stimulating in vitro the CD40 molecule on the membrane of CLL cell with the CD40 ligand the inefficient APC is turned into an efficient APC. In vivo, such an activation may conceivably occur in specific environments like the spleen and favour the production by normal residual B- cells of polyclonal autoAb against red cell or platelet self Ag.

Original languageEnglish
JournalHematology and Cell Therapy
Volume39
Issue numberSUPPL. 1
DOIs
Publication statusPublished - Nov 1997

Fingerprint

Autoimmunity
B-Lymphocytes
Autoantigens
Cytokines
T-Lymphocytes
Agammaglobulinemia
CD40 Ligand
Antigen-Presenting Cells
Autoantibodies
Signal Transduction
Blood Cells
Blood Platelets
Spleen
Macrophages
Cell Membrane
Antigens

Keywords

  • Antigen presenting cells
  • Autoimmunity
  • B cell receptor
  • Cytokines
  • Immunodeficiency

ASJC Scopus subject areas

  • Hematology

Cite this

Relationship between autoimmunity and immunodeficiency in CLL. / Caligaris-Cappio, F.

In: Hematology and Cell Therapy, Vol. 39, No. SUPPL. 1, 11.1997.

Research output: Contribution to journalArticle

Caligaris-Cappio, F. / Relationship between autoimmunity and immunodeficiency in CLL. In: Hematology and Cell Therapy. 1997 ; Vol. 39, No. SUPPL. 1.
@article{e2bca4d669f04cbea87377bbe80847a8,
title = "Relationship between autoimmunity and immunodeficiency in CLL",
abstract = "B-CLL patients have a high prevalence of autoimmune phenomena due to polyclonal autoantibodies (Abs) restricted to blood cell self-antigens (Ag) and progressively develop a severe hypogammaglobulinemia. Autoimmunity and immunodeficiency have been related to B-CLL biological properties and cellular origin. Three major issues have emerged: 1) the relevance of B cell receptor abnormalities. More specifically, the importance of the defective expression of CD79b which may explain the faint to virtually undetectable amounts of monoclonal sig, the anomalous signal transduction and the failure to present antigens (Ag). 2) the possibility that the relentless accumulation of B-CLL cells is also under microenvironmental influences. These are brought about by the interplay of cytokines produced through cell/cell contacts among malignant B cells, macrophages and T cells. 3) the view that the very properties of accumulating anergic self-reactive CD5+ B lymphocytes may provide a bridge between autoimmunity and immune incompetence. CD5+ malignant B cells influence the cytokine production by T cells, but are inefficient Ag presenting cells (APC). As such they are a hurdle to the production of normal Ab. However, by stimulating in vitro the CD40 molecule on the membrane of CLL cell with the CD40 ligand the inefficient APC is turned into an efficient APC. In vivo, such an activation may conceivably occur in specific environments like the spleen and favour the production by normal residual B- cells of polyclonal autoAb against red cell or platelet self Ag.",
keywords = "Antigen presenting cells, Autoimmunity, B cell receptor, Cytokines, Immunodeficiency",
author = "F. Caligaris-Cappio",
year = "1997",
month = "11",
doi = "10.1007/s00282-997-0013-8",
language = "English",
volume = "39",
journal = "Hematology and Cell Therapy",
issn = "1269-3286",
publisher = "Springer Verlag",
number = "SUPPL. 1",

}

TY - JOUR

T1 - Relationship between autoimmunity and immunodeficiency in CLL

AU - Caligaris-Cappio, F.

PY - 1997/11

Y1 - 1997/11

N2 - B-CLL patients have a high prevalence of autoimmune phenomena due to polyclonal autoantibodies (Abs) restricted to blood cell self-antigens (Ag) and progressively develop a severe hypogammaglobulinemia. Autoimmunity and immunodeficiency have been related to B-CLL biological properties and cellular origin. Three major issues have emerged: 1) the relevance of B cell receptor abnormalities. More specifically, the importance of the defective expression of CD79b which may explain the faint to virtually undetectable amounts of monoclonal sig, the anomalous signal transduction and the failure to present antigens (Ag). 2) the possibility that the relentless accumulation of B-CLL cells is also under microenvironmental influences. These are brought about by the interplay of cytokines produced through cell/cell contacts among malignant B cells, macrophages and T cells. 3) the view that the very properties of accumulating anergic self-reactive CD5+ B lymphocytes may provide a bridge between autoimmunity and immune incompetence. CD5+ malignant B cells influence the cytokine production by T cells, but are inefficient Ag presenting cells (APC). As such they are a hurdle to the production of normal Ab. However, by stimulating in vitro the CD40 molecule on the membrane of CLL cell with the CD40 ligand the inefficient APC is turned into an efficient APC. In vivo, such an activation may conceivably occur in specific environments like the spleen and favour the production by normal residual B- cells of polyclonal autoAb against red cell or platelet self Ag.

AB - B-CLL patients have a high prevalence of autoimmune phenomena due to polyclonal autoantibodies (Abs) restricted to blood cell self-antigens (Ag) and progressively develop a severe hypogammaglobulinemia. Autoimmunity and immunodeficiency have been related to B-CLL biological properties and cellular origin. Three major issues have emerged: 1) the relevance of B cell receptor abnormalities. More specifically, the importance of the defective expression of CD79b which may explain the faint to virtually undetectable amounts of monoclonal sig, the anomalous signal transduction and the failure to present antigens (Ag). 2) the possibility that the relentless accumulation of B-CLL cells is also under microenvironmental influences. These are brought about by the interplay of cytokines produced through cell/cell contacts among malignant B cells, macrophages and T cells. 3) the view that the very properties of accumulating anergic self-reactive CD5+ B lymphocytes may provide a bridge between autoimmunity and immune incompetence. CD5+ malignant B cells influence the cytokine production by T cells, but are inefficient Ag presenting cells (APC). As such they are a hurdle to the production of normal Ab. However, by stimulating in vitro the CD40 molecule on the membrane of CLL cell with the CD40 ligand the inefficient APC is turned into an efficient APC. In vivo, such an activation may conceivably occur in specific environments like the spleen and favour the production by normal residual B- cells of polyclonal autoAb against red cell or platelet self Ag.

KW - Antigen presenting cells

KW - Autoimmunity

KW - B cell receptor

KW - Cytokines

KW - Immunodeficiency

UR - http://www.scopus.com/inward/record.url?scp=0031422081&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0031422081&partnerID=8YFLogxK

U2 - 10.1007/s00282-997-0013-8

DO - 10.1007/s00282-997-0013-8

M3 - Article

VL - 39

JO - Hematology and Cell Therapy

JF - Hematology and Cell Therapy

SN - 1269-3286

IS - SUPPL. 1

ER -