XERODERMA PIGMENTOSUM: IMMUNOLOGICAL STUDIES IN THE PATIENTS
- VernacularTitle:着色性干皮病患者的免疫学研究
- Author:
Yeping TIAN
- Publication Type:Journal Article
- From:
Academic Journal of Second Military Medical University
1983;0(S1):-
- CountryChina
- Language:Chinese
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Abstract:
Studies on the immunologic function in patients with Xeroderma Pigmentosum (XP) were reported in this paper. The results showed that the patients had decreased rates of Et-and Ea-rosette formatian of T-lymphocytes;their lymphoblastic transformation ability to PHA were lower than normal individuals; 3 out of 4 patients showed negative OT tests except case 1 was positive; 3 patients gave negative reations to DNCB ( case 3 not tested ); the percentage of ANAE+ cells and SmIg+ cells were in normal range; the number of Ty cells increased in varying degree and most prominent in case 4, while the T? cell did not varied apparently except in case 4 which markedly decreased; the T?/ T? ratios were lower than normal. On the correlation analysis between the percentages of T?, T? or T?/T? ratio and levels of immunoglobulins showed that T? cell was significantly negative correlation with IgG and IgA, while T? cell was very significantly positive correlation with IgA; the T? / T? ratio was significantly positive correlation with IgG and IgA, but they had no correlation with lymphoblastic transformation. Besides T? cell gave significantly negative correlation with T? cell. No autoimmune antibody and circulating immuue complex were detected. It was found that there were aberrations of serum albumin, ?1,?2 and ?-globulins in varying degrees, especially the ?2 macro-globulins (?2M) were markedly increased in all 4 patients by immunoelectraphoretic assay, while ?-globulins were all in normal range. The total complement hemolytic unit (CH50) and C3 content were reduced in case 1 and case 2. The most striking feature was that the ability producing a type interferon (IFN-?) of leukocytes from all 4 XP patients was vary significontly lower than normal controls. Particularlly, bath the titer of IFN-ot produced and 3H-TdR uptake in lymphoblastic transformation of the isolated peripheral blood leukocytes or lymphocytes either prior to or after irradiation with different dosages of ultraviolet rays were all greatly lower in 4 XP patients than normal subjects. The difference of the percentage of IFN-? titer and lymphoblastic transformation rate decreased between XP patients and normal subjects was very sig-nificent. Judging by F test, it showed that there were very significent differences in IFN-? production ability and lymphoblastic transformation rate among different XP patients whose leukocytes or lymphocytes were under UV-irradiation. The decrease of above parameters was more marked in the patient with serious defect of DNA repair function than others, but no difference was seen in normal individuals. These results probably meant that the extent of decreasing IFN-? production and lymphoblastic transformation rate were corresponded with the degree of defect in DNA repair. Discussion on the decrease of immunologic functions seen in the XP patients has been made, which might be one of the important reasons of high cancer incidenc in this disease.
