A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease - PubMed (original) (raw)
Case Reports
A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease
M C Sneller et al. J Clin Invest. 1992 Aug.
Abstract
In mice, the two distinct autosomal recessive genes lpr and gld can induce a syndrome characterized by autoantibody formation and the progressive accumulation of an unusual CD4-CD8- T cell population in peripheral lymphoid tissue. This phenotype does not precisely mirror any human disease. In this report we describe two patients with a progressive lymphoproliferative disorder associated with autoimmunity. The peripheral blood and lymph nodes of these patients contained large numbers of an unusual CD4-CD8- T cell population. These CD4-CD8- T cells express surface markers characteristic of mature peripheral blood T cells (CD3, CD2, CD5), express the alpha/beta form of the T cell receptor, and do not express surface markers characteristic of immature thymocytes (CD1) or NK cells (CD16, CD56). Functionally, these cells exhibited deficient proliferation and lymphokine production upon stimulation with mitogenic antibodies to CD3 or CD2. Both proliferation and lymphokine production could be augmented by co-stimulation with an antibody directed at the CD28 determinant. The clinical and immunological features of this syndrome resemble the lymphoproliferative/autoimmune disease seen in lpr and gld mice.
Similar articles
- CD2 expression correlates with proliferative capacity of alpha beta + or gamma delta + CD4-CD8- T cells in lpr mice.
Budd RC, Russell JQ, van Houten N, Cooper SM, Yagita H, Wolfe J. Budd RC, et al. J Immunol. 1992 Feb 15;148(4):1055-64. J Immunol. 1992. PMID: 1346621 - Parallels in T lymphocyte development between lpr and normal mice.
Budd RC, Van Houten N, Clements J, Mixter PF. Budd RC, et al. Semin Immunol. 1994 Feb;6(1):43-8. doi: 10.1006/smim.1994.1007. Semin Immunol. 1994. PMID: 8167306 Review. - Gld and lpr mice: single gene mutant models for failed self tolerance.
Bhandoola A, Yui K, Siegel RM, Zerva L, Greene MI. Bhandoola A, et al. Int Rev Immunol. 1994;11(3):231-44. doi: 10.3109/08830189409061729. Int Rev Immunol. 1994. PMID: 7930847 Review.
Cited by
- Somatic mutations in FAS pathway increase hemophagocytic lymphohistiocytosis risk in patients with T- and/or NK-cell lymphoma.
Liu Y, Sardana R, Nemirovsky D, Frosina D, Jungbluth A, Johnson WT, Vardhana S, Arcila M, Horwitz SM, Derkach A, Dogan A, Xiao W. Liu Y, et al. Blood Adv. 2024 Jun 25;8(12):3064-3075. doi: 10.1182/bloodadvances.2023011733. Blood Adv. 2024. PMID: 38593227 Free PMC article. - Molecular and temporal control of restimulation-induced cell death (RICD) in T lymphocytes.
Lee KP, Epstein B, Lake CM, Snow AL. Lee KP, et al. Front Cell Death. 2023;2:1281137. doi: 10.3389/fceld.2023.1281137. Epub 2023 Oct 30. Front Cell Death. 2023. PMID: 38074985 Free PMC article. - Autoimmune lymphoproliferative immunodeficiencies (ALPIDs): A proposed approach to redefining ALPS and other lymphoproliferative immune disorders.
Magerus A, Rensing-Ehl A, Rao VK, Teachey DT, Rieux-Laucat F, Ehl S. Magerus A, et al. J Allergy Clin Immunol. 2024 Jan;153(1):67-76. doi: 10.1016/j.jaci.2023.11.004. Epub 2023 Nov 17. J Allergy Clin Immunol. 2024. PMID: 37977527 Review. - Genes and Microbiota Interaction in Monogenic Autoimmune Disorders.
Costa F, Beltrami E, Mellone S, Sacchetti S, Boggio E, Gigliotti CL, Stoppa I, Dianzani U, Rolla R, Giordano M. Costa F, et al. Biomedicines. 2023 Apr 8;11(4):1127. doi: 10.3390/biomedicines11041127. Biomedicines. 2023. PMID: 37189745 Free PMC article. Review. - Somatic mosaicism in inborn errors of immunity: Current knowledge, challenges, and future perspectives.
Aluri J, Cooper MA. Aluri J, et al. Semin Immunol. 2023 May;67:101761. doi: 10.1016/j.smim.2023.101761. Epub 2023 Apr 14. Semin Immunol. 2023. PMID: 37062181 Free PMC article. Review.
References
- J Exp Med. 1989 Mar 1;169(3):795-806 - PubMed
- Nature. 1989 Oct 5;341(6241):447-50 - PubMed
- FASEB J. 1987 Aug;1(2):103-9 - PubMed
- J Immunol. 1987 Mar 15;138(6):1660-6 - PubMed
- Nature. 1988 Jun 23;333(6175):742-6 - PubMed
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Research Materials