1.Relationship between initial absolute lymphocyte counts and the prognosis of children with primary immune thrombocytopenia
Ming'en LYU ; Yang LI ; Wenjie LIU ; Rongfeng FU ; Tiantian SUN ; Cuicui LYU ; Renchi YANG
Chinese Journal of Applied Clinical Pediatrics 2015;30(15):1147-1151
Objective To investigate the value of initial absolute lymphocyte counts (ALC) in predicting the prognosis of children with primary immune thrombocytopenia(ITP).Methods The initial clinical data of 214 children with incipient ITP were retrospectively analyzed and followed up for more than one year.Statistical analysis was made to find out the influence factors for the prognosis of patients with ITP in children to evaluate the predictive value of ALC on assessing the prognosis of ITP in children,and to analyze the association between ALC and outcomes and the clinical value of prognostic stratification.Results The remission rate of children with ITP was 71.5% in 12 months.Multivariate unconditioned Logistic analysis showed that disease duration,the infection history,response to treatment and ALC were independent risk factors for chronic ITP(all P < 0.05).Cutoff value of initial ALC was 3.005 × 109/L,sensitivity and specificity of value were 71.9%,83.6%.Three months and 12 months no remission rate of ITP in children between ALC > 3.005 × 109/L and ≤ 3.005 × 109/L had statistical significant difference (24.2% vs 64.9%,8.3% vs 54.2%,x2 =42.13,P <0.001).There was no statistical difference in therapy efficacy between 2 groups(x2 =5.098,P > 0.05).Remission rate between the ≤5 years old group and > 5 years old group was statistical difference based on age stratification(x2 =22.371,22.177,all P < 0.01).ITP in children were stratified into low risk group,intermediate risk group,high risk group based on the initial ALC and infection history,with remission rates in 3 month respectively 75.8%,54.1%,22.8%,respectively,and 91.6%,68.4%,31.6% in 12 months,respectively;there were statistically significant differences (x2 =44.867,68.802,all P < 0.001).Conclusions Initial ALC is an independent effecting factor in the prognosis of children with ITP.Initial ALC could be used for stratifying patients with the infection history before 6 weeks and supposed to be a predictive index for the prognosis of ITP in children.
2. The incidence and risk factors for thrombosis in primary immune thrombocytopenia
Yueting HUANG ; Xiaofan LIU ; Yunfei CHEN ; Rongfeng FU ; Wei LIU ; Feng XUE ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2018;39(11):942-946
Objective:
To probe the incidence and risk factors for thrombosis in Chinese immune thrombocytopenia through a retrospective analysis of the inpatients referred to the Blood Disease Hospital, CAMS & PUMC.
Methods:
A retrospective survey of 3 225 patients with ITP from October 2005 to December 2017 was performed, the clinical data of the patients with thrombosis were collected to analyze the causes, diagnosis, treatment and prognosis.
Results:
A total of 46 patients experienced a thrombotic event with a prevalence of 1.43%(46/3 225 cases) with the median age of thrombosis as 54 years (26-83) years, the prevalence of thrombosis was 3.37% (40/1 187 cases) in>40 years old, which was significantly higher than 0.58% (6/1 030 cases) in those under 40 years old, in adults (
3. The efficacy and safety of eltrombopag in Chinese patients with chronic immune thrombocytopenia
Yueting HUANG ; Xiaofan LIU ; Yunfei CHEN ; Rongfeng FU ; Wei LIU ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2018;39(1):32-36
Objective:
To investigate the safety and efficacy of eltrombopag for adult patients with chronic immune thrombocytopenia (cITP).
Methods:
It was a randomised, single-centre, 6 weeks, placebo-controlled study. Beginning in January 29th, 2013, 35 patients were enrolled, and the trial was completed on May 16th, 2014. 17 patients were assigned to receive eltrombopag (starting dose 25 mg/d) and 18 were assigned to receive placebo.
Results:
A total of 35 cases of adult cITP, 6 males and 29 females with a median age of 42(22-66) years were enrolled. One patient withdrew from eltrombopag treatment group for the adverse event (AE) and discontinued treatment. In first two weeks, 27.78% (5/18) of placebo-treated compared with 64.71%(11/17) of eltrombopag-treated patients achieved platelet counts ≥ 30×109/L(
4. Clinical evaluation of Chinese disseminated intravascular coagulation scoring system (version 2017) in patients with acute promyelocytic leukemia
Yueting HUANG ; Xiaofan LIU ; Rongfeng FU ; Yunfei CHEN ; Wei LIU ; Feng XUE ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2018;39(6):480-484
Objective:
To evaluate the applicability of Chinese disseminated intravascular coagulation scoring system (CDSS) in the diagnose of DIC in patients with acute promyelocytic leukemia (APL) patients.
Methods:
Medical records of 220 APL patients diagnosed and receiving induction therapy in Blood Disease Hospital, CAMS & PUMC from January 2004 to February 2018 were retrospectively analyzed. Each patient was evaluated by CDSS, the International Society of Thrombosis and Haemostais (ISTH) scoring system for overt DIC and Japanese Ministry of Health and Welfare (JMHW) scoring system for overt DIC, respectively.
Results:
A total of 220 APL patients were enrolled in the study, with a median age of 38.5 (12-70) years, 114 male and 106 female. Among them, 173 were in the low-medium risk group, 47 high-risk group; 11 patients died during induction treatment. The positive rates of DIC diagnosed by CDSS criteria, ISHT criteria, JMHW criteria was 62.27%, 54.09%, 69.09%, respectively. The consistency rate of CDSS and ISTH in diagnosing DIC was 78.10%; the consistency rate of CDSS and JMHW was 88.32%. There was significant difference in PT, APTT, FIB, D-Dimer and FDP in DIC(+) and DIC(-) group by CDSS (all P<0.05), but patients in the DIC(+) group had lower level of D-Dimer than in the DIC(-) group [21.9(1.2-477.1) mg/L
5. Immune tolerance induction in a case of hemophilia B with inhibitor with prothrombin complex concentrate and rituximab
Feng XUE ; Wei LIU ; Yunfei CHENG ; Xiaofan LIU ; Yueting HUANG ; Rongfeng FU ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2017;38(9):749-753
Objective:
To explore the immune tolerance induction (ITI) in a case of severe hemophilia B patient with inhibitor.
Methods:
The F Ⅸ∶C was detected using a one-stage method and FIX inhibitor was assayed using Bethesda method. ITI was performed with prothrombin complex concentrates (PCC) in combination with rituximab.
Results:
His past exposure days (ED) with PCC were 20 ED and his peak FⅨ inhibitor titer was 56 BU/ml. When his FIX inhibitor titer decreased to 10.4 BU/ml in Nov. 2015 and after receiving the informed consent from his parents, ITI was started. PCC with low dose rituximab successfully eradicated the high titer inhibitor within 17 months. There was no anaphylaxis, thrombotic event and infection.
Conclusion
This is the first case report for successful immune tolerance induction therapy in Chinese hemophilia B patient. ITI using PCC combined with rituximab is an effective choice to induce immune tolerance of hemophilia B with inhibitor.
6. Clinical evaluation of the revised International Prognostic Score of Thrombosis for essential thrombocythemia (IPSET-thrombosis) in a cohort of 746 Chinese adult patients
Rongfeng FU ; Huiyuan LI ; Feng XUE ; Xiaofan LIU ; Wei LIU ; Yueting HUANG ; Yunfei CHEN ; Liyan ZHANG ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2017;38(2):92-96
Objective:
To evaluate the role of the revised International Prognostic Score of Thrombosis (IPSET-thrombosis) in predicting the occurrence of thrombotic events in Chinese patients with essential thrombocythemia (ET) and to develop a thrombosis predicting model more applicable to Chinese ET patients.
Methods:
Medical records of 746 adult patients with an initial diagnosis of ET were retrospectively analyzed.
Results:
The median age at diagnosis was 52 (18-87) years, with 305 males and 441 females. According to the revised IPSET-thrombosis model, the number of very low-, low-, intermediate-, and high-risk patients were 271 (36.3%) , 223 (29.9%) , 63 (8.4%) and 189 (25.3%) , respectively. The four groups exhibited significantly different thrombosis-free survival (
7. Predictors of fatigue among individuals with primary immune thrombocytopenia in China
Yang LI ; Ming’en LYU ; Yating HAO ; Boyang SUN ; Yueting HUANG ; Rongfeng FU ; Feng XUE ; Xiaofan LIU ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2017;38(5):384-389
Objective:
To study the fatigue symptoms of adult patients with primary immune thrombocytopenia (ITP) and to analyze the possible factors that affect the severity of fatigue.
Methods:
Eligible adult patients with ITP who admitted to Institute of Hematology & Blood Diseases Hospital were enrolled in this study and the questionnaires including a Chinese version of the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) , the Pittsburgh sleep quality index (PSQI) , the Hospital Anxiety and Depression scale (HADS) and demographic information were completed. The predictors of fatigue were determined with multiple linear regression analyses.
Results:
A total of 207 patients with ITP were enrolled, including 70 males (33.8%) and 137 females (66.2%) , the median age was 42 (18-72) years old. The FACIT-F score in ITP patients was (37.50±9.05) . The FACIT-F severity of ITP patients was positively correlated with the platelet count (
8.Lupus anticoagulant: two cases report and literature review.
Yang LI ; Ming' en LYU ; Feng XUE ; Wenjie LIU ; Yating HAO ; Yue GUAN ; Boyang SUN ; Cuicui LYU ; Xueping GU ; Rongfeng FU ; Yueting HUANG ; Wei LIU ; Yunfei CHEN ; Xiaofan LIU ; Lei ZHANG ; Renchi YANG
Chinese Journal of Hematology 2016;37(2):130-133
OBJECTIVETo deepen the understanding of clinical manifestations and treatment of patients with positive lupus anticoagulant (LAC).
METHODSThe clinical data of 2 patients were analyzed and related literature were reviewed.
RESULTSCase 1, a 31-year-old female, diagnosed as lupus anticoagulant positive, secondary to undifferentiated connective tissue disease, was presented with menorrhagia and thrombocytopenia. Anti-nuclear antibody (ANA) was positive 1:1000 (homogeneous type) with anti-double stranded DNA positive, and dRVVT LA1/LA2 was 3.4. Coagulation function was alleviated after treatment with glucocorticoid and total glucosides of paeony. Case 2, a 59-year-old female was presented with gingival bleeding, hematuria with the level of F II:C 13%. dRVVT LA1/LA2 was 2.0. Anti-nuclear antibody (ANA) was positive 1:1000 (type of cytoplasmic granule), anti-double stranded DNA was positive. The patient was diagnosed as hypoprothrombinemia-lupus anticoagulant syndrome (LAHS) and acquired coagulation factor deficiency. The signs of hemorrhage were alleviated after treatment with methylprednisolone 40 mg/day and cyclophosphamide, while the level of F II:C was below normal.
CONCLUSIONSymptoms of patients with positive LAC are variable. The diagnosis relies on history of disease and laboratory test. Currently, there is no standardized treatment. Cases of LAHS should be thoroughly investigated for any known causes and related disorder.
Adult ; Blood Coagulation ; Cyclophosphamide ; therapeutic use ; Female ; Glucocorticoids ; therapeutic use ; Hematologic Tests ; Hemorrhage ; Humans ; Hypoprothrombinemias ; diagnosis ; Lupus Coagulation Inhibitor ; blood ; Methylprednisolone ; therapeutic use ; Middle Aged
9. Gene mutation spectrum and clinical characteristics analysis of 178 patients with essential thrombocytosis
Huan DONG ; Rongfeng FU ; Mankai JU ; Ting SUN ; Xiaofan LIU ; Feng XUE ; Ying CHI ; Renchi YANG ; Lei ZHANG
Chinese Journal of Hematology 2019;40(10):837-842
Objective:
To analyze the gene mutation spectrum, clinical features, and the factors of disease progression and prognosis in patients with essential thrombocytosis (ET) .
Methods:
A retrospective analysis was conducted on 178 newly diagnosed ET patients admitted from February 1st, 2009 to November 1st, 2018.
Results:
Of the 178 patients, 89 were male and 89 female, and the median diagnosis age was 49.5 (3-86) years old. JAK2V617F, CALR and MPL mutations frequencies were 16.45% (1.67%-43.90%) , 40.00% (10.00%-49.15%) and 25.10% (25.00%-40.00%) , respectively. Compared with patients with CALR mutations, patients with JAK2V617F mutation had higher diagnosis age (
10. A phase Ⅲ multi-center clinical trial on safety and efficacy of a domestic plasma derived factor Ⅸ for the treatment of patients with hemophilia B
Wei LIU ; Rongfeng FU ; Yawei ZHOU ; Yun CHEN ; Jie YIN ; Ziqiang YU ; Linhua YANG ; Meifang WANG ; Hui BI ; Zeping ZHOU ; Xinsheng ZHANG ; Jielai XIA ; Renchi YANG
Chinese Journal of Hematology 2018;39(5):404-407
Objective:
To evaluate the efficacy and safety of a domestic human plasma derived coagulation Factor Ⅸ concentrate (pd-FⅨ) in patients with hemophilia B.
Methods:
The study was a multicenter, open-label and single-arm study. The efficacy of pd-F Ⅸ was evaluated by objective performance criteria. The doses of pd-FⅨ were calculated according to the bleeding symptom and disease severity. The infusion efficiency of pd-FⅨ and improvement of bleeding symptoms were measured at 30 minutes and (24±4) h after the first infusion, respectively. Adverse events were recorded. Viral infection and FⅨ inhibitor were detected 90 d after the first infusion.
Results:
All 36 subjects with hemophilia B were enrolled in the study. The median age of these patients was 31 years old and the median injection doses were 4 (1-17) times. The hemostatic effect of 27/36 (75.00%) and 9/36 (25.00%) acute bleeding events were rated as "excellent" and "better" , respectively. The recovery rate was 111.92% (65.55%-194.28%) at 30 minutes after infusion of FⅨ. There was no adverse event related to FⅨ. No reactivation of HBV, HCV or HIV and FⅨ inhibitor was detected at 90-104 d after the first FⅨ infusion.
Conclusion:
This domestically made human plasma derived FⅨ concentrate is safe and effective in the treatment of acute bleeding in patients with hemophilia B.
Clinical trial registration
China food and Durg Administration, 2016L08027.