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混合性冷球蛋白血症时的极度高补体血症。

Extreme hypercomplementemia in the setting of mixed cryoglobulinemia.

机构信息

Division of Rheumatology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.

出版信息

Clin Rheumatol. 2011 Mar;30(3):415-8. doi: 10.1007/s10067-010-1652-7. Epub 2011 Jan 7.

DOI:10.1007/s10067-010-1652-7
PMID:21213005
Abstract

Elevated levels of complement components, C3 and C4, are often seen in inflammatory diseases. They are generally not thought to be responsible for organ injury but simply reflect stimulation of the immune system. Extremely high elevations of these components are rarely seen. Such values are usually reported in patients with hematopoietic malignancies. We report here a case of C3 elevation to 9.22 g/L (5 times the upper limit of normal) and C4 elevation to 2.48 g/L (6 times the upper limit of normal). To our knowledge, no prior elevations of this magnitude have been reported previously from our institution or in the published medical literature (+/- MEDLINE search, using keywords hypercomplementemia, C3, C4, and lymphoplasmacytic lymphoma). In this case, there appeared to be no adverse end-organ damage from the excessive complement components or their activation.

摘要

补体成分 C3 和 C4 水平升高常见于炎症性疾病。一般认为它们不会导致器官损伤,而只是反映免疫系统受到刺激。这些成分的极高水平很少见。此类值通常在造血系统恶性肿瘤患者中报告。我们在此报告一例 C3 升高至 9.22g/L(正常值上限的 5 倍)和 C4 升高至 2.48g/L(正常值上限的 6 倍)。据我们所知,此前我们机构或已发表的医学文献中均未报告过如此大幅度的升高(+/- 使用关键词 hypercomplementemia、C3、C4 和 lymphoplasmacytic lymphoma 进行 MEDLINE 搜索)。在本例中,似乎没有来自过量补体成分或其激活的不良终末器官损伤。

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本文引用的文献

1
Rituximab in cryoglobulinaemic vasculitis, evidence for its effectivity: a case report and review of literature.利妥昔单抗治疗冷球蛋白血症性血管炎的疗效分析:一例报告及文献复习
Clin Rheumatol. 2011 Feb;30(2):293-300. doi: 10.1007/s10067-010-1612-2. Epub 2010 Oct 31.
2
Reference distributions for complement proteins C3 and C4: a practical, simple and clinically relevant approach in a large cohort.补体蛋白C3和C4的参考分布:在一个大型队列中的实用、简单且与临床相关的方法
J Clin Lab Anal. 2004;18(1):1-8. doi: 10.1002/jcla.10100.
3
Cryoglobulinemia based on interaction between a gamma macroglobulin and 7S gamma globulin.
基于γ巨球蛋白与7Sγ球蛋白相互作用的冷球蛋白血症。
Am J Med. 1962 Jan;32:142-7. doi: 10.1016/0002-9343(62)90191-2.
4
Hepatic response to sepsis: interaction between coagulation and inflammatory processes.肝脏对脓毒症的反应:凝血与炎症过程之间的相互作用。
Crit Care Med. 2001 Jul;29(7 Suppl):S42-7. doi: 10.1097/00003246-200107001-00016.
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Mixed cryoglobulinemia as a possible preneoplastic disorder.
Arthritis Rheum. 1995 Dec;38(12):1859-60. doi: 10.1002/art.1780381222.
6
Hypercomplementaemia as a marker of the evolution from benign to malignant B cell proliferation in patients with type II mixed cryoglobulinaemia.
Br J Rheumatol. 1994 Aug;33(8):791-2. doi: 10.1093/rheumatology/33.8.791.
7
The complement system in essential mixed cryoglobulinemia.
Ric Clin Lab. 1980 Jan-Mar;10(1):75-80. doi: 10.1007/BF02984908.
8
Immune complexes and complement profile in essential mixed cryoglobulinemia before and after plasma exchange.
Int J Artif Organs. 1983 Jul;6 Suppl 1:65-8.
9
Complement activation and impaired capacity to solubilize immune complexes or to prevent their formation in essential mixed cryoglobulinemia.补体激活以及在原发性混合性冷球蛋白血症中溶解免疫复合物或阻止其形成的能力受损。
Diagn Immunol. 1983;1(4):315-23.
10
Serum complement levels in patients with mixed (IgM-IgG) cryoglobulinaemia.混合性(IgM-IgG)冷球蛋白血症患者的血清补体水平。
Clin Exp Immunol. 1966 Jul;1(3):337-9.