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14例朗格汉斯细胞组织细胞增多症的免疫学发现。

Immunological findings in 14 cases of Langerhans cells histiocytosis.

作者信息

Martín Mateos M A, Muñoz López F, Monferrer R, Cruz M

机构信息

Servicio de Inmunología y Alergia, Hospital Clínico, Facultad de Medicina, Universidad de Barcelona, Spain.

出版信息

J Investig Allergol Clin Immunol. 1991 Oct;1(5):308-14.

PMID:1688293
Abstract

We have studied humoral and cell immunity in 14 children, from 2 months to 15 years old, diagnosed with Langerhans cells histiocytosis who had not received immunosuppressant nor immunopotentiating treatment. At the time of diagnosis, children were found in the following Greenberger clinical stages: stage II (5), stage III (6), stage IV (3). The children in stage II had moderate alterations in immunity, with an increase in IgG, IgM, IgA, increase in T4 lymphocytes and decrease in T8 lymphocytes; the clinical evolution was good. In stage III, normal immunoglobulins were noted in 2 children, an increase in IgG, IgM, IgA in 1; an increase in IgM in 1, a decrease in IgM in 1 and absence of IgA in 1. In all a decrease was found in T8 lymphocytes and the T4 lymphocytes were decreased in 3 and normal in 3; delayed immune response and response to mitogens was decreased in 3. These findings were related to a bad prognosis; 2 children died and another developed chronic disease. In stage IV serious alterations were detected in the immune system with a decrease in IgG, IgM, IgA, an increase in IgE, a decrease in T, B, T4 and T8 lymphocytes and very slight delayed immune response and response to mitogens; the children died.

摘要

我们研究了14名年龄从2个月至15岁、诊断为朗格汉斯细胞组织细胞增多症且未接受过免疫抑制或免疫增强治疗的儿童的体液免疫和细胞免疫。在诊断时,这些儿童处于以下格林伯格临床分期:II期(5名)、III期(6名)、IV期(3名)。II期儿童的免疫功能有中度改变,表现为IgG、IgM、IgA升高,T4淋巴细胞增多,T8淋巴细胞减少;临床病程良好。在III期,2名儿童的免疫球蛋白正常,1名儿童的IgG、IgM、IgA升高,1名儿童的IgM升高,1名儿童的IgM降低,1名儿童无IgA。所有患儿的T8淋巴细胞均减少,3名患儿的T4淋巴细胞减少,3名患儿的T4淋巴细胞正常;3名患儿的迟发性免疫反应和对有丝分裂原的反应降低。这些发现与预后不良有关;2名儿童死亡,另1名儿童发展为慢性病。在IV期,免疫系统出现严重改变,表现为IgG、IgM、IgA降低,IgE升高,T、B、T4和T8淋巴细胞减少,迟发性免疫反应和对有丝分裂原的反应非常轻微;这些儿童均死亡。

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