Abstract

Objectives To investigate distribution of T lymphocyte subsets, absolute value and proportion of large granular lymphocyte(LGL) in peripheral blood and bone marrow of acquired pure red cell aplasia (PRCA) patients, and to evaluate patients′ immune function and efficacy of immunosuppressive agents. Methods From January 2002 to November 2015, a total of 25 patients with acquired PRCA who were treated at Department of Hematology, People′s Hospital of Xinjiang Uygur Autonomous Region at first-visit were enrolled into this study, as study group. Inclusion criteria of study group: patients′ diagnoses were conformed to the diagnostic criteria of PRCA in Diagnosis and Curative Effect of Hematology (3rd edition) and clinical data were complete. Exclusion criteria: other anemia disease and clinical data were not complete. In 25 cases of PRCA patients, 5 cases were secondary PRCA with T cell-large granular lymphocyte leukemia (T-LGLL), other 20 cases were primary PRCA. And 25 cases of health people who were receiving physical examination at the People′s Hospital of Xinjiang Uygur Autonomous Region during the same period were recruited into control group randomly, by simple random method. Inclusion criteria of control group: no abnormality in blood routine examination and biochemical test. Exclusion criteria: abnormality in blood routine examination, and suffering from immune disease, tumor. T lymphocyte subsets of subjects in study group and control group were detected by flow cytometry. Blood routine examination and bone marrow examination were carried out in study group before and after treatment. Then absolute value and proportion of LGL in bone marrow, peripheral blood and curative effect after drug treatment of cyclosporine A and so on of 25 acquired PRCA patients were analyzed retrospectively and compared statistically. The study protocol was approved by the Ethical Review Board of Investigation in Human at People′s Hospital of Xinjiang Uygur Autonomous Region. There were no statistically significant differences of subjects′ clinical data, such as age and gender ratio, between two groups (P>0.05). Results ①Compared with control group, helper T lymphocyte (Th) proportion and Th/suppressor T lymphocyte (Ts) ratio of acquired PRCA patients in study group were significantly lower, Th proportion: (36.6±3.8)% vs (45.1±2.1)%; Th/Ts ratio: 1.2±0.2 vs 2.2±0.4; there were significant differences (t=9.161, 12.174, P=0.032, 0.021), and Ts proportion of study group was significantly higher than that of control group, (30.5±2.8)% vs (20.2±1.9)%; there was significant difference (t=13.460, P=0.021). There was no statistically significant difference of natural killer (NK) cell proportion between two groups, (11.3±1.8)% vs (10.3±1.3)%; and t=1.572, P=0.344. In study group, Th proportion, Th/Ts ratio of secondary PRCA patients with T-LGLL were lower than those of primary PRCA patients, Th proportion: (36.1±3.7)% vs (39.1±6.1)%; Th/Ts ratio: 1.0±0.2 vs 1.2±0.1; but the differences were not significant (t=2.293, 2.513; P=0.301, 0.297), and Ts proportion of secondary PRCA patients with T-LGLL was higher than that of primary PRCA patients, (31.0±2.7)% vs (28.4±2.0)%; but the difference was not significant (t=1.472, P=0.384). There was no statistically significant difference of NK cells proportion between two groups, (11.3±1.8)% vs (11.1±2.7)%; and t=1.572, P=0.364. ② Among 25 acquired PRCA patients in study group, 16 cases had higher proportion of lymphocytes in peripheral blood (64%, 16/25), and 10 cases had 2~3 granules LGL (40%, 10/25); 8 cases had more than 20% LGL of total lymphocytes (32%, 8/25). Four cases had 2~3 granules LGL in bone marrow (16%, 4/25); 3 cases had more than 20% LGL of total lymphocytes (12%, 3/25). The ratio of patients with LGL in peripheral blood was significantly higher than that in the bone marrow (χ2=6.595, P=0.003). ③ After treatment, hemogram of 18 cases returned to normal, including four cases with normal myelogram, fourteen cases with decreased bone marrow erythroid cell hyperplasia, which was higher than that before treatment. Effectiveness evaluation of study group was as follows: four PRCA patients were cured basically; fourteen cases achieved remission; six cases achieved significant progress; one case was invalid. The total effective rate of treatment was 96% (24/25). Five secondary PRCA patients with T-LGLL didn′t need blood transfusion after treatment, but unlike primary PRCA patients, their hemoglobin levels didn′t recover to normal reference range. Conclusions The disorder of T lymphocyte subsets in patients with acquired PRCA caused the disorder of cellular immune function, and LGL detected in peripheral blood would help the diagnosis of acquired PRCA. Effect of cyclosporin A treatment on acquired PRCA was very significant, but curative effect of secondary PRCA patients with T-LGLL was poorer than that of primary PRCA. Key words: Red-cell aplasia, pure; Anemia, aplastic; T-lymphocyte subsets; Leukemia, large granular lymphocytic; Cyclosporine

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