A NOVEL LYMPHOPROLIFERATIVE AUTOIMMUNE SYNDROME RESEMBLING MURINE LPR/GLD DISEASE

被引:245
作者
SNELLER, MC
STRAUS, SE
JAFFE, ES
JAFFE, JS
FLEISHER, TA
STETLERSTEVENSON, M
STROBER, W
机构
[1] NCI, PATHOL LAB, BETHESDA, MD 20892 USA
[2] NIH, CTR CLIN, IMMUNOL SERV, BETHESDA, MD 20892 USA
关键词
AUTOIMMUNITY; DOUBLE-NEGATIVE; HUMAN; T-CELL RECEPTOR ALPHA/BETA CHAINS;
D O I
10.1172/JCI115867
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
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.
引用
收藏
页码:334 / 341
页数:8
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