Abstract:

Objective　To investigate the application significance of capillary electrophoresis analyzer in the diagnosis of hemoglobin(Hb) CS-H disease. Methods　A total of 34 patients with Hb CS-H disease confirmed by thalassemia genetic analysis and 70 patients with non Hb CS-H disease (including 42 cases of -^SEA/-α^3.7,19 cases of -^SEA/-α^4.2,7 cases of Hb WS-H and 2 cases of Hb QS-H) were determined for Hb with Sebia Capillarys 2 capillary electrophoresis. The thalassemia genetic analysis of the 2 groups was conducted bygap-polymerase chain reaction (Gap-PCR) and reverse dot blot hybridization. Results　The HbCS and HbH contents of Hb CS-H group were (1.89±1.33)% and (1.07±0.86)%,and the HbA₂ content was (1.17±0.68)%. However,the samples with HbCS and a part of HbH were not detected in non-Hb CS-H group,and the HbA₂ content was (1.81±1.21)%. Compared with Hb CS-H group and non-Hb CS-H group for HbA₂,there were significant significance (P<0.05). Compared with the thalassemia genetic analysis,the sensitivity of capillary electrophoresis for the diagnosis of Hb CS-H disease was 88.2%,the specificity was 100.0%,the positive predictive value was 100.0%,the negative predictive value was 94.6%,the diagnosis efficiency was 96.2%,and there was no statistical significance (P=0.134). Conclusions　The capillary electrophoresis can be used to the rapid diagnosis of Hb CS-H disease,and it will alleviate some of the economic burdens for patients.

Key words: Capillary electrophoresis, Thalassemia, Hemoglobin CS-H disease, Hemoglobin disease