CLINICAL AND IMMUNOLOGICAL ANALYSES OF 103 PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY
CLINICAL AND IMMUNOLOGICAL ANALYSES OF 103 PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY
复制标题
DOI：
10.1007/bf00917124
复制
发表时间：
1989-01-01
影响因子：
9.1
通讯作者：
CUNNINGHAMRUNDLES, C
中科院分区：
文献类型：
--
作者：
CUNNINGHAMRUNDLES, C
共同变量免疫缺陷(CVI)或低丙种球蛋白血症是一种异质性的原发免疫缺陷疾病，其中B细胞产生很少或不产生抗体。由于这种疾病相对罕见，相关疾病的范围很广，患者得到了各种专家的护理。因此，很难确定具体并发症的发生率。在这些研究中，我们分析了103名连续转诊的CVI患者，他们的年龄范围为3-71岁(平均29岁)，随访时间为1-13年(总计750个患者年)。初诊时未经治疗的患者血清免疫球蛋白平均为174.4 mg/dl，肌注免疫球蛋白治疗的患者为301 mg/dl。免疫球蛋白治疗前后平均IgA为14.5，平均IgM为80.7，差异无统计学意义。大约一半的患者有T细胞功能障碍，但淋巴细胞刺激反应与年龄呈负相关，这意味着随着年龄的增长，T细胞免疫功能下降。血清Ig G和Ig A水平呈正相关(P=0.008.0 1)，且血清Ig G水平与刀豆蛋白A刺激淋巴细胞有关(P=0.0 1)。截至1986年，79例患者存活，23例死亡，1例下落不明。复发性细菌性疾病对所有患者都很常见，22%的患者发展为慢性肺部疾病，22%的患者患有自身免疫性疾病，15%的患者患有癌症，13%的患者患有肝炎，9%的患者吸收不良。自身免疫性疾病在女性中更常见，癌症更有可能在第五和六十年发展。在11%的患者中，其他家庭成员被发现患有免疫缺陷(低丙种球蛋白血症或IgA缺陷)。9名患者死于呼吸功能不全(有或无其他并发症)，7名患者死于癌症。这些数据提供了关于免疫异常以及与低丙种球蛋白血症相关的疾病的谱和频率的有价值的信息。
Common variable immunodeficiency (CVI) or hypogammaglobulinemia is a heterogeneous primary immunodeficiency disease in which B cells produce little or no antibody. Since the disease is relatively rare and the spectrum of associated illnesses is broad, patients are given care by a variety of specialists. Thus it has been difficult to determine the incidence of specific complications. In these studies we analyzed 103 consecutively referred CVI patients of age range 3-71 years (average, 29 years) who were followed for a period of 1-13 years (total of 750 patient years). The average serum IgG was 174.4 mg/dl for untreated patients and 301 mg/dl for patients treated with intramuscular immunoglobulin at the time of the first visit. The average IgA was 14.5, and the average IgM was 80.7, with no difference between or after immunoglobulin treatment. About one-half of the patients had T-cell dysfunction, but lymphocyte stimulation responses were inversely related to age, which implies worsened T-cell immunity with age. Serum IgG and IgA levels were found to be statistically associated (P = 0.008), and serum IgG was related to lymphocyte stimulation with concanavalin A (P = 0.01). By 1986, 79 patients were alive, 23 had died, and 1 could not be located. Recurrent bacterial illnesses were common to all patients, and 22% had developed chronic lung disease, 22% autoimmune disease, 15% cancer, 13% hepatitis, and 9% malabsorption. Autoimmune disease was more common in females, and cancer was more likely to develop in fifth and sixth decades. In 11% of the group, other family members were found to be immunodeficient (hypogammaglobulinemic or IgA deficient). Nine patients died of respiratory insufficiency (with or without other complications), and seven patients died of cancer. These data provide valuable information about the immunologic abnormalities and the spectrum and frequency of illnesses associated with hypogammaglobulinemia.