[Clinical characteristics and outcome of 69 patients with thrombotic thrombocytopenic purpura].

To analyze the clinical characteristics, diagnosis, treatment and outcome of patients with thrombotic thrombocytopenic purpura (TTP). The clinical data of 69 adult patients with TTP were retrospectively analyzed. There were 19 males and 50 females with a median age of 42 (18-79) years. PLASMIC score 6-7 was recognized in 82.8% (53/64) patients. The activity of von Willebrand factor-cleaving protease (ADAMTS13), which was detected in 21 patients before treatment, was less than 5% in 17 patients and 5%-10% in 3 patients. All 69 patients were treated with plasma exchange (PEX) and/or fresh frozen plasma infusion (PI), 43 of whom were also given glucocorticoid. In addition to PEX/PI and glucocorticoid, rituximab and/or immunosuppressants were administrated in 20 patients. The median follow-up time was 12 (1-57) months. The remission rate was 69.6%, while the relapse rate was 11.6%. The 2-year overall survival (OS) rate was 69.6%±5.5%. The univariate and multivariate analysis showed that relapsed/refractory disease was an independent risk factor for OS. The 2-year OS rate of relapsed/refractory patients was significantly lower than that of the rest patients (41.5%±9.8% vs. 83.7%±5.6%, P<0.001). Regarding the unfavorable prognosis in relapsed/refractory patients, rituximab and/or immunosuppressants are strongly recommended for sake of improving the overall survival.

[Prognostic significance of different IDH mutations and accompanying gene mutations in patients with acute myeloid leukemia].

Objective: To investigate the prognostic significance of different IDH mutations and accompanying gene mutations in patients with non-M(3) acute myeloid leukemia (AML) . Methods: Second-generation sequencing was performed to detect the mutations of 22 genes in 389 patients with AML in the First Affiliated Hospital of Zhengzhou University from June 2016 to December 2018, and Kaplan-Meier and Cox regression models were used to analyze the prognostic factors. Results: The mutation frequency of IDH1 and IDH2 was 6.2% and 8.7% , respectively, in all patients without co-mutation. The IDH2 mutant group had an older age, higher proportion of bone marrow primitive cells, more common normal karyotype, and more common RUNX1 and SRSF2 mutations compared with IDH2 wild-type group. Univariate analysis of variance showed that the median OS and PFS of IDH1 mutation group were significantly shorter than those of the wild-type group (P<0.05) . IDH2 mutation as a single variable and IDH2R140 mutation had no significant effect on the prognosis, while different mutation sites had different effects. Compared with the IDH2 wild-type group, the IDH2R172 mutation group had lower complete remission (CR) rate and shorter median OS and PFS (P<0.05) . In patients with normal karyotypes or aged ≥50 years, IDH2 mutation as a single variable had no significant effect on the prognosis, IDH1 mutation and IDH2R172 mutation were associated with poor OS and PFS (P<0.05) , and IDH2R140 mutation had no significant effect on OS and PFS. Approximately 74.1% (43/58) of patients with IDH mutation simultaneously carried other gene mutations; however, the number of accompanying gene mutations had no significant effect on the prognosis. Among 58 patients with IDH mutation, the CR rate of patients with NPM1 mutation was significantly higher than that of patients in the NPM1 wild-type group (81.8% vs 36.4% , P=0.014) , the median OS in patients with DNMT3A mutation was lower than that of patients with DNMT3A wild type [4.0 months (95% CI 3.8-4.2) vs 6.3 months (95% CI 2.4-10.2) , P=0.041) ]. Multivariate analysis showed that age ≥60 years and white blood cell count ≥100×10(9)/L were independent risk factors for OS and PFS, while CR after two courses of treatment and hematopoietic stem cell transplantation were independent prognostic favorable factors for OS and PFS. Conclusion: In patients with AML (non-M(3)) , IDH gene mutations often coexisted with other gene mutations, and different subtypes and accompanying gene mutations of IDH have different prognostic significance.

[Clinical features and prognostic factors in adult acute myeloid leukemia patients with FLT3-ITD and CEBPA gene co-mutation].

Objective: To investigate the clinical characteristics and prognosis in adult acute myeloid leukemia (AML) patients with FLT3-ITD and CEBPA double-mutated (CEBPAdm) co-mutation. Methods: Clinical data and prognostic factors were retrospectively analyzed in adult AML patients with FLT3-ITD and CEBPAdm co-mutation at The First Affiliated Hospital of Zhengzhou University from January 2016 to September 2018. Results: Among 599 non-acute promyelocytic leukemia (APL) patients, 268 received gene mutation detection, who were divided into 4 groups including 19 FLT3-ITD positive (FLT3-ITD(+)) and CEBPAdm positive (CEBPAdm(+)) cases (group A) , 84 FLT3-ITD(+) and CEBPAdm(-) cases (group B) , 95 FLT3-ITD(-) and CEBPAdm(+) cases (group C) , 70 double negative mutation cases (group D) . Gender, platelet count, FAB classification, induction treatment regimen and fusion gene mutation were comparable among four groups (P>0.05) , while age onset, peripheral white blood cell (WBC) count, hemoglobin, percentage of blasts in peripheral blood, percentage of blasts in bone marrow, complete remission rate (CR(1) rate) after the first induction chemotherapy, the relapse rate, the median progression-free survival (PFS) time, and median overall survival (OS) time were significantly different between groups (P<0.05) . When compared in pairs, gender, age onset, hemoglobin, platelet count, FAB classification in group A were not statistically different compared to group B, C and D (P>0.05) , while patients in group A had higher WBC count, blasts in peripheral blood, minimal residual disease (MRD) in bone marrow. The CR(1) rates of group A, B, C, and D were 50.0%、32.4%、59.8%、39.0% respectively (P=0.003) , and the relapse rates were 55.6%, 50.0%, 21.1%, 40.0% (P<0.001) . As to survival, the median OS in each group was 6.25, 3.0, 15.5, 10.5 months respectively (P<0.001) , and the median PFS was 5.0, 4.0, 10.0, 6.7 months (P=0.032) . Conclusion: Adult AML patients with FLT3-ITD and CEBPAdm co-mutation have a higher leukemia load and low CR(1) rate, which translates into poor prognosis with high relapse rate and short survival time.

[The clinical characteristics and prognosis of 20 patients with H7N9 avian influenza].

Objective: To analyzed the clinical characteristics and prognosis of patients with H7N9 avian influenza. Methods: The baseline characteristics, clinical manifestations, treatments, laboratory and imaging findings were collected and analyzed for 20 patients with H7N9 avian influenza admitted to the First Affiliated Hospital of Nanchang University from December 2016 to March 2017. According to the final clinical outcome, the patients were divided into the death group and the survival group. Ten patients in the death group died, and 10 patients in the survival group were discharged. The data with normal distribution were analyzed by t test. The data with non-normal distribution were analyzed by Wilcoxon rank sum test. Results: Of the 20 patients, 13 were males and 7 were females, aging 40-82 years, with a mean age of (60±12) years. Twelve patients had a definite history of poultry exposure and 10 had chronic underlying diseases such as hypertension and diabetes. The clinical manifestations were mainly fever, cough, hemoptysis, respiratory distress, fatigue, etc. In the survival group, the platelet count was(167-315)×10(9)/L, while it was (78-152)×10(9)/L in the death group. The average white blood cell count was (7.78-11.52)×10(9)/L and (9.91-15.93)×10(9)/L in the survival and death groups respectively. The average value of lymphocyte count was (0.69-1.59)×10(9)/L and (0.58-0.86)×10(9)/L in the survival and death groups respectively. In the death group the glutamic-pyruvic transaminase (ALT) value was (14.0-352.0) U/L, the total bilirubin value was (6.9-34.5) µmol/L, the creatine kinase MB (CK-MB) was (16.0-162.0) U/L, the serum calcium value was (1.4-2.0) mmol/L, the C-reactive protein value was (33.1-414.0) mg/L, and the calcium reduction prime value was (0.6-100.0) µg/L. In the survival group,the ALT value was (25.0-181.0) U/L, the total bilirubin value was (4.8-25.9) µmol/L, the CK-MB value was (15.0-40.0) U/L, the serum calcium value was (1.9-2.4) mmol/L, the C-reaction protein value was (12.8-52.5) mg/L, and the procalcitonin value was (0.3-23.3) µg/L. Sixteen cases suffered severe pneumonia. Twelve patients received extracorporeal membrane oxygenation (ECMO), and 4 survived. The cause of death was mainly related to factors such as age, chronic underlying diseases and severity of illness. Conclusions: Human infection with H7N9 avian influenza virus was highly pathogenic, and prone to progress into severe pneumonia, with a high mortality. Decreased platelet count was associated with mortality (t=4.07, P=0.001), predictive of patient outcome.

[Comparison of different styles of allogeneic hematopoietic stem cell transplantation as first-line treatment treated with severe aplastic anemia in children and adolescents].

Objective: To evaluate the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) from different donors as first-line treatment for children and adolescents with severe aplastic anemia (SAA) . Methods: The clinical data of 79 children and adolescents with SAA diagnosed from January 2013 to December 2016 in Henan Province were retrospectively analyzed. There were 50 males and 29 females, with a median age of 14(4-18) years. 40 cases received matched sibling transplantation (MSD-HSCT), 17 with unrelated donor transplantation (UD-HSCT), and 22 with haploidentical transplantation (haplo-HSCT). Results: The comparison of MSD-HSCT, UD-HSCT, haplo-HSCT groups was conducted and the median times of neutrophils engraftment were statistically significant [12(9-25) d, 14(10-22) d, 16(11-26) d, respectively (χ2=13.302, P=0.001)], but no difference in+30 d engraftment rate [97.3%(36/37), 100%(15/15), 100%(20/20), χ2=0.959, P=0.619]. The median times of PLT engraftment were not statistically significant [14(6-34)d, 16(7-32)d, 19(10-34)d, respectively, χ2=5.892, P=0.053], and the +30 d engraftment rate had no difference [97.3%(36/37), 100%(15/15), 100%(20/20), χ2=0.959, P=0.619]. The post-transplant infection rate showed no statistically significance [35.0% (14/40), 29.4% (5/17), 45.5% (10/22), χ2=1.158, P=0.560], as well as the incidences of aGVHD, grade III/IV aGVHD and cGVHD(χ2=0.230, P=0.891; χ2=2.628, P=0.269; χ2=3.187, P=0.203). The two-years OS rate was not statistically significant respectively [(77.1±6.7)%, (70.6±11.1)%, (77.3±8.9)%, χ2=0.330, P=0.845]. Severe post-transplant infection (RR=4.617, P=0.009), grade Ⅲ/Ⅳ aGVHD (RR=2.707, P=0.048) were independent risk factors for OS. Conclusion: The overall efficacy of MSD-HSCT, UD-HSCT and haplo-HSCT as first-line therapy for children and adolescents with SAA/VSAA is comparable.

Modified busulfan and cyclophosphamide conditioning regimen for allogeneic hematopoietic stem cell transplantation in the treatment of patients with hematologic malignancies.

OBJECTIVE: We aim to evaluate the clinical efficacy of a modified busulfan and cyclophosphamide (BU/CY) conditioning regimen for allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of hematologic malignancies. METHODS: A total of 45 patients with hematologic malignancies were treated using stem cell transplantation between March 2007 and June 2012. All the patients received a modified BU/CY conditioning regimen before transplantation. The outcomes of the patients were followed up including mortality, survival, relapse, and complications. RESULTS: The median of follow-up duration was 527 days. All the patients who received modified BU/CY conditioning regimen achieved hematopoietic recovery successfully. Among the patients, 24 were survived without complications, 5 had relapsed hematologic malignancies, and 16 died. The median time to leucocyte engraftment was 14 days and to platelet engraftment was 12 days. Acute graft-versus-host disease (aGVHD; grades I-IV) occurred in 15 patients (30%). The cumulative incidence of grades I aGVHD was 22.2% (10 patients), grades II was 6.7% (3 patients), and grades III-IV was 4.4% (2 patients). Among 40 appreciable patients, 8 (20%) developed chronic GVHD. The incidence rate of hemorrhagic cystitis and veno-occlusive disease were 15.5% and 2.2%, respectively. CONCLUSIONS: The modified BU/CY conditioning regimen for allo-HSCT is effective and safe for the treatment of hematologic malignancies.

[Effect and nervous mechanism of acupuncture on gastroelectrical propagation velocity].

20 fasting cats were used to investigate the propagation of Gastroelectrical Propagation Velocity (GPV) and the effect and nervous mechanism of acupuncture on it. The results are as follow: 1. GPV can be described as V = bo - b1x1 + b2x2. bo, b1 and b2: regression coefficient. x1: frequency of slow-wave. x2: amplitude of slow-wave. 2. GPV is changeable on normal condition, and it can be affected by stimulation of vagi or splantic nerve and by administration of pentagastrin i.v. or atropine i.v. with some change of not only x1, x2 but also b0, b1, b2. 3. Manual acupuncture and electrical acupuncture can affect GPV differently. 4. After vagotomy the effect of manual acupuncture on GPV was changed. All results suggest that GPV can be affected by acupuncture with complex mechanism including the change of propagation resistance and of propagation refractory period.

[Influence of acupuncture on discharge of gastric signal vagal efferent fiber].

16 fasting cats were used to investigate the effect of acupuncture on discharge of gastric signal vagal efferent fibers (GSVEF). 1. The discharge of GSVEF may increase or decrease while artificially increasing intragastric pressure. 2. Both manual acupuncture and electronic acupuncture could enhance or decrease GSVEA's discharge. All results suggest that acupuncture may adjust gastric activity through affecting gastric vago-vagal reflex.