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.

Objective:To investigate the efficacy and safety of zanubrutinib combined regimens for treatment of elderly patients with central nervous system lymphoma (CNSL).Methods:The clinical data of 3 elderly patients with CNSL who received zanubrutinib combined regimens from July 2020 to March 2022 in the Affiliated Suzhou Hospital of Nanjing Medical University were retrospectively analyzed. Two of them were primary central nervous system lymphoma (PCNSL) and one was secondary central nervous system lymphoma (SCNSL). Relevant literature was reviewed at the same time.Results:The two patients with PCNSL were treated with zanubrutinib in combination with rituximab and lenalidomide (Z-R 2) regimen. One of them achieved complete remission (CR) after 6 cycles, and the other patient achieved partial remission (PR) after 1 cycle of Z-R 2 regimen. The patient with SCNSL was diagnosed with diffuse large B-cell lymphoma (DLBCL) previously and suffered from central nervous system relapse after achieving CR. The patient achieved PR after 2 cycles of Z-R 2 regimen, and then achieved CR followed by 4 cycles of zanubrutinib in combination with rituximab and high-dose methotrexate (Z-R-HD-MTX) regimen. Two of the three patients were still in CR until April 2022, and the longest disease-free survival time was 15 months. There were no severe adverse reactions during the treatment. Conclusions:Zanubrutinib combined regimens for 3 elderly patients with CNSL have shown promising effect and well tolerance, which may be considered as novel treatment for CNSL patients who are unable to tolerate severe chemotherapies or autologous hematopoietic stem cell transplantations.

Objective To summarize the clinical effect and adverse reactions of avatrombopag combined with tacrolimus in the treatment of elderly patient with refractory severe aplastic anemia.Methods An elderly patient with refractory severe aplastic anemia was treated with avatrombopag and tacrolimus,and the literature review on the mechanism of action and safety of avatrombopag and ta-crolimus was conducted.Results The patient was treated with a combination of cyclosporine,testos-terone undecanoate,and itraconazole for 6 months without success,and developed liver and kidney dysfunction.The medication was discontinued,and the patient was treated with a combination of avatrombopag(20 mg,once per day)and tacrolimus(1 mg,twice per day).After 9 months of treat-ment,the patient's platelet count increased to 54 × 109/L,the hemoglobin increased to 124 g/L,the white blood cells increased to 8.23 × 109/L,liver and kidney function also returned to normal level,and no other adverse reactions such as thrombotic events were observed.After treatment,CD4/CD8 de-creased,CD8+T cells increased,and the proportion of Treg cells increased as well.Conclusion The combination of avatrombopag and tacrolimus is effective and well tolerated in the treatment of elderly patient with refractory aplastic anemia,but the long-term follow-up observation and continued accumu-lation of cases are still needed in the future.

Objective To summarize the clinical effect and adverse reactions of avatrombopag combined with tacrolimus in the treatment of elderly patient with refractory severe aplastic anemia.Methods An elderly patient with refractory severe aplastic anemia was treated with avatrombopag and tacrolimus,and the literature review on the mechanism of action and safety of avatrombopag and ta-crolimus was conducted.Results The patient was treated with a combination of cyclosporine,testos-terone undecanoate,and itraconazole for 6 months without success,and developed liver and kidney dysfunction.The medication was discontinued,and the patient was treated with a combination of avatrombopag(20 mg,once per day)and tacrolimus(1 mg,twice per day).After 9 months of treat-ment,the patient's platelet count increased to 54 × 109/L,the hemoglobin increased to 124 g/L,the white blood cells increased to 8.23 × 109/L,liver and kidney function also returned to normal level,and no other adverse reactions such as thrombotic events were observed.After treatment,CD4/CD8 de-creased,CD8+T cells increased,and the proportion of Treg cells increased as well.Conclusion The combination of avatrombopag and tacrolimus is effective and well tolerated in the treatment of elderly patient with refractory aplastic anemia,but the long-term follow-up observation and continued accumu-lation of cases are still needed in the future.

OBJECTIVETo explore the clinical significance, reliability and responsiveness of ITPBAT bleeding grading system for patients with immune thrombocytopenia (ITP).

METHODSOne hundred and eighty-three patients with ITP were assessed by using of ITP-BAT bleeding grading system. Test-retest reliability, responsiveness of ITP-BAT bleeding grading system and association between bleeding grades and platelet counts, age, gender, disease stage were analyzed.

RESULTSBleeding degree of ITP patients and the platelet count were negatively correlated (r=- 0.744, P<0.01) and bleeding degree increased significantly with platelet counts below 20×10⁹/L (χ²=82.40，P<0.01). Mild bleeding rate in children was 68.5%, higher than that in adult(χ²=8.839，P<0.01), and severe bleeding rate in the elderly was 14.3%, higher than that in non-elderly(χ²=7.056，P<0.01). There were no significant differences in bleeding degree in patients with different gender and disease stage (χ²=4.922, P>0.05 and χ²=3.411, P>0.05). Bleeding grades before and after treatment had more significant difference(Z=-6.61, P<0.01). Scoring consistency of two doctors was 66.1% (κ=0.561), and scoring consistency of the same doctor was 94.7% (κ=0.874).

CONCLUSIONITP-BAT bleeding grading system in China has good validity and responsiveness, closely related to clinical indicators. It is sensitive to the variation of the hemorrhage in patients. ITP-BAT could be used as a reference index of the treatment, and also be used as an observation index of curative effect.

Hemorrhage ; etiology ; physiopathology ; Humans ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; complications ; physiopathology ; Reproducibility of Results

OBJECTIVETo explore incidence, risk factors and prognosis of the first 6 months infectious events in adults with newly diagnosed primary immune thrombocytopenia (ITP), and evaluate the value of initial absolute lymphocyte count (ALC) in predicting infection.

METHODSThe initial clinical records and infectious events during 6 months of 217 adult with newly diagnosed ITP were retrospectively analyzed. Statistical methods were used to analyze risk factors of the 6 months infections in adults ITP, the prediction of ALC in risk of infection, and the association of ALC and prognosis.

RESULTSInfection rate of ITP patients accepting therapy within 6 months after the initial diagnosis was 13.8% (30/217), and infection rate of patients ≥ 60 years of age 25% (14/56). Multivariate unconditioned Logistic analysis showed that gender and ALC were independent risk factors for the 6 months infection of ITP patients (P<0.05, 95% CI 1.150-7.298, OR 2.722 and P<0.001, 95% CI 6.802-80.749, OR 23.436). Cutoff value of ALC was 1.225 × 10⁹/L, sensitivity and specificity of its value were 0.866 and 0.700 respectively. Infection rate of ALC>1.225 × 10⁹/L in adult ITP was lower than of ALC ≤ 1.225 × 10⁹/L (5.3% vs 45.7%, χ² = 49.151, P<0.001). Furthermore, persistent recovery and the 1-year mortality rate after diagnosis had no difference among patients of different ALC (28.0% vs 26.0%, χ² = 0.071, P>0.05, and 98.6% vs 97.8%, χ² = 0.095, P>0.05). There were no significant differences in persistent recovery in patients with and without infection (30.0% vs 27.3%, χ² = 0.096, P>0.05). The 1-year mortality rate after diagnosis was significantly lower in those patients who developed an infection (93.3% vs 99.3%, χ² = 4.607, P<0.05).

CONCLUSIONInitial ALC was an independent risk factor of 6 months infection in adult ITP. It could be a predictive index of infection within 6 months of the initial diagnosis in ITP patients. Infection as an important factor affected the survival of ITP patients.

Adult ; Humans ; Lymphocyte Count ; Middle Aged ; Multivariate Analysis ; Prognosis ; Purpura, Thrombocytopenic, Idiopathic ; Retrospective Studies ; Risk Factors

OBJECTIVETo explore the clinical value of immature platelet fraction (IPF), absolute immature platelet fraction (A- IPF) and thrombelastograph (TEG) on assessment of bleeding risk of immune thrombocytopenia (ITP).

METHODStwo hundred and seventy- one patients with ITP were assessed based on ITP-BAT bleeding grading system. IPF, A-IPF were determined in 271 patients ,TEG in 125 patients. The correlations between bleeding grades and IPF, A-IPF, variables of TEG in subgroups were analyzed by statistical method. The predictive value of IPF, A-IPF, and variables of TEG on bleeding risk of ITP patients was evaluated.

RESULTSThere were no significant differences in bleeding degree in all patients with different gender and disease stage (P>0.05). Mild bleeding rate in children was higher than that in adult (P<0.05). PLT inversely correlated with bleeding grade for the entire cohort (P<0.001). In all subjects, PLT< 30 × 10⁹/L and pediatric cohorts with PLT< 30 × 10⁹/L, PLT were negatively correlated with IPF (P<0.05), positive correlated with A-IPF (P<0.001) and the maximum amplitude (MA (P<0.05). Bleeding grades were significantly correlated with IPF, A-IPF, MA in all subjects and patients with PLT< 30 × 10⁹/L (P<0.001). IPF, A-IPF and MA did not correlate with bleeding grades in children with PLT< 30 × 10⁹/L (P>0.05). ROC curve analysis revealed IPF, A-IPF and MA had better predictive value (AUC 0.745, 0.744, 0.813, P<0.001). Multivariate analysis showed that IPF and MA were independence factors for predicting bleeding risk in ITP patients and comprehensive predictive value was higher (AUC 0.846, P<0.001) than single variable.

CONCLUSIONIPF, A-IPF and MA could accurately evaluate bleeding risk in ITP patients. It may be considered as reference index of the treatment and observation index of curative effect.

Adult ; Blood Platelets ; Child ; Hemorrhage ; etiology ; physiopathology ; Humans ; Multivariate Analysis ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic ; complications ; physiopathology ; ROC Curve