3.2.1 Improvement of anemia and transfusion dependence
The anemia of 17 patients (17/20, 85.00%) significantly improved. Fourteen patients (14/20, 70.00%) were able to stop blood transfusion, and 3 patients (3/20, 15.00%) had hemoglobin levels that recovered to normal. Among the 6 patients (6/20, 30.00%) who still needed blood transfusion, the interval time of blood transfusion was prolonged from 6 days (Hb 48 g/L) to 19 d (Hb 59 g g/L) in 3 patients. Generally, anemia improved, and hemoglobin increased from 68.40 ±14.79 g/L to 86.80 ± 24.32 g/L (p = 0.0042) (Fig. 2A1, 2A2). Among the 20 patients, 3 patients (3/20, 15.00%) had no significant improvement in anemia (HB fluctuated between 40-50 g/L) and still needed intermittent blood transfusion. Eculizumab will hopefully be available in China in the near future. It is believed that this drug could improve the clinical symptoms of the two patients for whom chemotherapy was ineffective.
3.2.2 The hemolytic symptoms were controlled, and the hemolytic indexes were obviously improved .
The effect of chemotherapy on chronic intravascular hemolysis is characterized by a continuous decrease in LDH levels. The mean LDH levels decreased from 1592.0 ± 718.7 U/L at baseline to 390.0 ± 252.8 U/L at 8 weeks (p = 0.002) (Fig. 2C1, 2C2). In addition, TBIL and the percentage of reticulocytes were evaluated as indirect biochemical indicators of intravascular hemolysis. After chemotherapy, reticulocytes decreased from 10.20 ± 7.01% to 4.23 ± 3.03% (p=0.0012) (Fig. 2B1, 2B2). Of the 20 patients, 3 patients (3/20, 15.00%) had normal TBIL, and the remaining 17 patients had a significant decrease in TBIL from 41.42 ± 22.92 µmol/L to 18.72±9.08 µmol/L (p=0.003) (Fig. 2D1, 2D2). Plasma free hemoglobin and haptoglobin levels were monitored in another 12 patients. The results showed that the serum free hemoglobin concentration was normal in 2 patients, and the decrease was significant in the other patients. Not all patients were tested for the above indicators, so they were not included in the analysis.