Basic characteristics of patients with TTP:
In this study, a total of 114 patients with TTP were studied. In terms of sex distribution, 80 cases (70.2%) were female and 34 cases (29.8%) were male. The mean age was 39.3 + 14.9 years ranging from 21 to 84 years. In total, 83 patients (72.8%) were outpatient and 31 cases (27.2%). In terms of disease classification, 47 (41.2%) were idiopathic, 21 (18.4%) pregnancy-type, 31 (27.2%) immunologic, 7 The case (6.1%) was followed by transplantation, in 6 cases (5.3%) it was cancerous and in 2 cases (1.8%) it was drug induced. In terms of disease manifestations, hematologic manifestations in 64 cases (56.1%), neurologic manifestations in 24 cases (21.1%), routine laboratory manifestations in 22 cases (19.3%), jaundice in 2 cases (1.8%) %), Fever in 1 case (0.9%) and increased creatinine level in 1 case (0.9%). In terms of laboratory presentation, mean hemoglobin was 9.58 ± 8.65 g / dl, mean creatinine was 1.77 ± 1.55 mg / dl, mean LDH level was 189.89 ± 1293.77 mg / dl. , Mean Schistosite count was 5.03 /1 1.27, mean platelet count was 36491.23 + 25836.53 mm3, mean AST level was 60.41+56.24 mg / dl, mean ALT level was 94.64+-2.76 mg / dl, mean total bilirubin level was 2.76 +2.29 mg / dl and mean direct bilirubin level was 2.76 ± 2.28 mg / dl. Mean reticulocyte count was 5.43 + 3.79. In the treatment protocol, all patients underwent PEX over an average of 16.61 rounds. Also, 12 (10.5%) patients received rituximab. Three patients received vincristine in total.
3.2.2 Outcome information for patients with TTP:
In terms of response to treatment and recovery, complete remission was reported in 72 cases (63.2%), partial remission in 14 cases (12.3%) and no remission in 28 cases (24.6%). On this basis, the total recovery rate after treatment was estimated to be 86 (75.5%). The incidence of recurrence was estimated to be 24 (21.1%) in total. Overall, the prevalence of mortality was 30 (26.3%). Mean CR was 31.17 ±31.34 months, mean platelet count normalization time was 15.24 ±18.42 days and mean recurrence time was 10.56 + 28.57 months.
- Calculating the survival of patients with TTP:
Based on Kaplan-Meier curve analysis of survival, 30-day survival was 82.5%, 76.3% one-year survival, 74.6% two-year survival, and 73.7% three-year survival. Five years was 2.71%. In terms of recurrence-free survival, 6-month recurrence-free survival was 93.8%, one-year recurrence-free survival equal to 89.3%, two-year recurrence-free survival equal to 89.3%, and three-year recurrence-free survival equal to 6. 85.5% and five-year recurrence-free survival were 2.83%.
Determination of Predictors of Mortality in Patients:
In both groups, the frequency of death was 60 (71.4%) and 20 (66.7%), respectively (P value = 0.625). The mean age of the two groups was 34.89 +10.14 years and 51.67 + 19.17 years, respectively, which was significantly higher in the deceased group (P value less than 0.001). According to the distribution of disease category in the two groups, the prevalence of idiopathic type was 39 cases (46.4%) and 8 cases (26.7%) respectively, the frequency of pregnancy type was 20 cases (23.8%). ) And 1 case (3.3%), frequency of immunological type was 20 cases (23.8%) and 11 cases (36.7%), frequency of transplant type was 3 cases (3.6%) and 4 cases (3 cases). (13%), malignant type was 0 (0%) and 6 (20%) and the frequency of drug type was 2 (2.4%) and 0 (0%), respectively. There was a significant difference between the two groups in that the frequency of malignancy was significantly higher in the deceased group (P value less than 0.001). In terms of clinical manifestations, the incidence of neurologic manifestations was 12 (14.3%) and 10 (40.0%), respectively (55.5%) in both groups. And 9 cases (30%), jaundice occurrence 0 (0%) and 2 cases (6.7%), fever occurrence 0 (0%) and 1 case (3.3) %), Laboratory presentation was 16 (19%) and 6 (20%) and creatinine increased 1 (1.2%) and 0 (0%), respectively, indicating higher prevalence. Neurologic manifestations were higher in patients (P value <0.001). In the group of deceased patients, mean hemoglobin was 8.13 +1.79 and 10/10 ± 9.98 (P value = 0.228), mean creatinine was 2.13 + 1.34 and 0.60, respectively. 1.6 1 1 (P value = 0.146), mean LDH was 2296/60+ 1513.03 and 2112.78 + 1213.83 (P value = 0.601), mean platelet count was 34100/00+29520/71(P value = 0.557), AST mean 50.5 ± 31.8 and 63.8 + 62.5 (P value 0.27), ALT mean 94.64 ±57.92 (P value 0.372), mean total bilirubin 2.76 ±1.88 and 2.76 ±2.31 (P value 0.986) The mean reticulocyte count was 4.2±3.4 and 5.8 ± 3.8 (P value = 0.034), which only showed a difference in reticulocyte count. The convenience was between the two groups. Ritoximab was 9 (11.0%) and 3 (11.1%), respectively, with no significant difference (p value = 0.945). According to Cox Proportion Hazard Model, two factors of high age (P value = 0.001) and appearance of neurological manifestations (P value of 0.001) were two predictors of long-term mortality in patients.
Determination of Predictors of Recurrence:
In the two groups with and without recurrence, the frequency of female was 20 (83.3%) and 60 (66.7%), respectively (P value = 0. 113). The mean age of the two groups was 32.46 9 9.54 years and 41.13 15 15.68 years, respectively, which was significantly lower in the relapsed group (P value = 0.011). According to the distribution of the disease in two groups with and without recurrence, the frequency of idiopathic type was 10 cases (41.7%) and 37 cases (41.1%) respectively, the frequency of pregnancy type was 2 cases (8.3%) and 19 cases (21.1%), frequency of immunologic type was 12 cases (50%) and 19 cases (21.1%), transplant type frequency equal to 0 cases (0.2%) and 7 cases (7.8%). %), Malignant type was 0 (0.2%) and 6 (6.7%), and drug-induced frequency was 0 (0.2%) and 2 (2.2%), respectively. There was a significant difference between the two groups in the frequency of relapsed immunologic type (P value = 0.042). In terms of clinical manifestations, the frequency of neurologic manifestations was 6 (25%) and 18 (20%) respectively, in the two groups with and without recurrence, hematologic involvement was 16 (66.7%) and 48 cases (53.3%), jaundice occurrence 0 (0.2%) and 2 cases (2.2%), fever occurrence 1 case (4.2%) and 0 cases (0.2%) Laboratory presentation was equal to 1 (4.2%) and 21 (23.3%) and creatinine increased to 0 (0.2%) and 1 (1.1%), respectively, indicating no difference between the two groups. Was (P value 0.10). In the group of patients with and without recurrence, mean hemoglobin was 8.26 +2.25 and 9.94 + 9.64 (P value 0.402), mean creatinine was 1.76 +1.91 and 1.45%, respectively. 1.78 (P value = 0.966), mean LDH was 1929.04 + 1929.04 and 2525.45 + 1361.20 (P value = 261.26), mean platelet count was 2106.61818.45 And 40611 ± 11/25993 82/82 (P value 0.001), AST mean 63.62 + 65.53 and 59.55 + 53.88 (P value 0.754), ALT mean 77.3+ 49.0 + 49.6 ± 99.0 (P value 0.609), mean total bilirubin 2.7 +2.5 and 2.7 +2.2 (P value 0.949) and mean The reticulocyte count was 7.9 + 4.9 and 6.7 + 3.1 (P value = 0.123), which only showed a difference in platelet count between the two groups. It was Roe. Ritoximab was 1 (4.3%) and 11 (12.8%), respectively, with no significant difference (p value 0.148). According to Cox Proportion Hazard Model, three factors of low age (P value 0.042), immunological category (P value 0.003) and decrease in platelet count (p value 0.001) are predictors of long-term recurrence. The duration was in patients. Table 1 lists the predictors of mortality TTP patient in our study.
Predictors of TTP Death
Factor
|
Beta multiplier
|
standard deviation
|
Probability ratio
|
P value
|
Older age
|
0.056
|
0.020
|
1.058
|
0.006
|
Malignant Cause
|
516/20-
|
611/1
|
0.001
|
0.999
|
Neurologic manifestation
|
588/1-
|
0.603
|
0.204
|
0.008
|
The reticulocyte count
|
102/0-
|
0.092
|
903/0
|
0.263
|
Table 1: Predictors of TTP Death in our study