This study aims to identify an optimal model for assessing the recurrence frequency of Budd–Chiari syndrome (BCS) and further analyze the factors contributing to BCS recurrence. A total of 754 patients who were admitted to the Affiliated Hospital of Xuzhou Medical University between January 2015 and July 2022 were included. We constructed 4 different count outcome models: Poisson, negative binomial (NB) model, zero-inflated Poisson (ZIP) model, and zero-inflated negative binomial (ZINB) model. We selected the model with the best fitting performance to explore factors associated with BCS recurrence. Of 754 respondents, 511 reported no recurrence. Log-likelihood ratio tests indicated that the NB model performed better than the Poisson regression model (χ 2 = 124.91, P < .001), and the ZINB model outperformed the ZIP model (χ 2 = 34.29, P < .001). In the ZINB model, the analysis of the counting process revealed that the variables significantly associated with recurrence frequency included age (odds ratio [OR] = 0.69; 95% confidence interval [CI]: 0.57–0.84), sex (female: OR = 1.77; 95% CI: 1.24–2.55), anticoagulant use (warfarin vs new oral anticoagulants [NOACs]: OR = 2.11, 95% CI: 1.34–3.31; not using anticoagulants vs NOACs: OR = 1.98, 95% CI: 1.20–3.28), absence of cirrhosis (OR = 0.57, 95% CI: 0.40–0.82), and neutrophil count (NEU) (OR = 1.22, 95% CI: 1.04–1.42). The zero-inflated model proved robust in identifying factors influencing BCS recurrence compared with the other models. Influence of sex, surgery, anticoagulation, cirrhosis, hospital duration, apolipoprotein A, and NEU on the recurrence risk and frequency of BCS.

Background: Little is known about the real-world survival benefits and safety profiles of carfilzomib-pomalidomide-dexamethasone (KPd) in patients with relapsed and refractory multiple myeloma (RRMM). Methods: We performed a retrospective multicenter analysis to evaluate the efficacy and safety of KPd regimen in 81 patients with RRMM registered in the Eastern Shandong Myeloma Collaborative Group. All patients received at least 1cycle of KPd. KPd was performed in RRMM patients as follows: intravenous carfilzomib at a dose of 20mg/m2 on days 1 and 2, and then 27mg/m2 on days 8, 9, 15, and 16 of the first cycle and days 1, 2, 8, 9, 15, and 16 of subsequent cycles, dexamethasone 20 mg on days 1, 2, 8, 9, 15, 16, 22, and 23, and pomalidomide 4 mg orally on days 1-21 of each 28-day cycle. Results: The median age at the start of KPd regimen was 63 years old (range, 38-74),39 were male and 42 were female. Among 81 patients included, 22.2% of them have received upfront autologous hematopoietic stem cell transplantation. Before patients received KPd regimen, there were 39 (48.2%),25 (30.9%) and 17 (20.9%) patients have already received prior 1-line, 2-line, and ≥ 3-line treatment, respectively. 91.4% of patients previously exposed to bortezomib (45.7% refractory), 63.0% exposed to lenalidomide (35.8% refractory), and 32.1% exposed to anti-CD38 monoclonal antibody (16.1% refractory). 63.0% of patients exposed to both bortezomib and lenalidomide (25.9% double refractory), and 24.7% exposed to bortezomib, lenalidomide and CD38 antibody (3.7% triple refractory). There were 36(44.4%) and 31(38.3%) patients with ISS stage III and extramedullary disease, respectively. 30.9% of patients had prior history of high blood pressure and 21.0% of patients had a history of cardiovascular disease (coronary disease, heart failure, arrhythmia, etc.). The median duration of treatment was 3 months. Among 69 patients available for response assessment, the objective response rate (ORR) (≥partial response) was 66.5%(n=46) with 42.0% achieving ≥very good partial response and 31.9% achieving≥complete response. After a median follow-up of 6 months, he median progression-free survival (PFS) and overall survival (OS) were 12.7 months (95%CI: 6.83, Not estimable) and not reached (95% CI: 11.17, Not estimable), respectively. A total of 79 adverse events were observed in 33(40.7%) KPd cases and Grade I, Grade II and Grade III events were accounted for 36.7%(n=29),40.5% (n=32) and 22.8%(n=18), respectively. Grade III events were only observed in 12 patients and no higher events were observed. Infections(n=5), cardiovascular(n=5) and cytopenia(n=3) events were most frequent reported Grade III events, no patients died due to KPd related adverse events. Conclusion: KPd regimen showed considerable clinical benefits among RRMM in a real-world setting, and the adverse effects after KPd are controllable.

AbstractObjective: To explore the effect of Philadelphia chromosome karyotype (Ph) and allogeneic hematopoietic stem cell transplantation (allo-HSCT) on the treatment of acute lymphoblastic leukemia (ALL).METHODS:The data of 429 patients with all from January 2012 to December 2020 were retrospectively analyzed. According to the results of cytogenetic karyotype analysis, they were divided into Ph+ group (n=64), Ph- monomeric karyotype (MK) group (n=53) and Ph- NMK group (n=312). According to the treatment plan, they were divided into allo-HSCT group (n=236) and non-allo-HSCT group (n=193). The effects of karyotype and allo-HSCT on the short-term and long-term outcomes of all patients were analyzed.RESULTS:Among the 429 patients, 6 (1.40%) died during induction therapy, 60 (13.99%) had no response, 363 (84.62%) achieved complete remission (CR) and 287 (66.90%) achieved minimal residual disease negative (MRD-). There was no significant difference in short-term efficacy (CR%, CR1%, MRD-%) among Ph+ group, Ph- MK group and Ph- non-MK group (P>0.05). The median OS was 6.9 months (95% CI: 4.6-8.2 months) for 60 unresponsive patients and 39.8 months (95% CI: 28.6-45.9 months) for 363 CR patients. There was no significant difference in the long-term efficacy ［5-year cumulative recurrence rate (CIR%), disease-free survival rate (DFS%) and overall survival rate (OS%) among Ph- group, Ph- MK group and Ph- non-MK group (P>0.05). Among 429 patients, 55.01% (236/429) underwent allo-HSCT. The short-term efficacy (CR%, MRD-%) and long-term efficacy (CIR%, DFS%, OS%)］ of patients with allo-HSCT after more than 2 consolidation cycles were better than those of patients with non-allo-HSCT (P<0.05). For the three subgroups of Ph+ group, Ph- MK group and Ph- non-MK group, the short-term and long-term efficacy of allo-HSCT patients was better than that of non-allo-HSCT patients. Multivariate logistic regression analysis showed that liver/spleen/lymph node enlargement was a risk factor for CIR, DFS and OS, with adjusted or of 1.23 (95% CI: 1.08-2.78, P=0.032), 1.21 (95% CI: 1.03-2.34, P=0.038) and 1.25 (95% CI: 1.08-2.97, P=0.028), respectively. No transplantation was a risk fator for CIR, DFS, OS. The adjusted or were 2.34 (95% CI: 1.18-5.39, P<0.001), 2.15 (95% CI: 1.10-4.34, P<0.001) and 2.28 (95% CI: 1.09-4.11, P<0.001), respectively.CONCLUSION:Karyotype (Ph+/- and MK/non-MK) seems to have no effect on the short-term and long-term efficacy of all patients; allo-HSCT can affect the short-term and long-term efficacy of all patients and improve their prognosis; liver/spleen/lymph node enlargement and non-implementation of allo-HSCT treatment strategy are the risk factors for poor prognosis of all patients.

OBJECTIVE:To investigate the application effect of sequential autologous hematopoietic stem cell transplantation (Auto-HSCT) with lenalidomide, bortezomib and dexamethasone (RVD) in the treatment of multiple myeloma (MM) evaluated by propensity score matching. METHODS:The clinical data of 49 MM patients treated with RVD scheme and followed-up for 36 months in the hospital from January 2015 to January 2021 were retrospectively analyzed and included in the control group, the clinical data of 54 MM patients who received RVD scheme and sequential Auto-HSCT scheme and completed 36 months of follow-up in the hospital during the same period were collected and included in the observation group. PSM method (1∶1, caliper value=0.01) was used to match the control group with the observation group based on baseline data and laboratory indexes, covariate equilibrium samples were obtained between groups (40 cases in each group). The clinical efficacy of patients in the two groups after 18 weeks of treatment was compared; the incidence of toxic and side effects during treatment of patients in the two groups was compared; the survival of patients in the two groups was compared after 36 months of follow-up. RESULTS:The ORR and DCR in the observation group were higher than those in the control group, the difference was statistically significant (P<0.05). Compared the incidence of fatigue, rash, thrombocytopenia, anemia and nausea of patients in the two groups, there was no statistical significant difference (P>0.05). After 36 months of follow-up (no loss during follow-up), 4 cases died from illness in the observation group, with a survival rate of 90% and an average survival time of 35.61 (95% CI: 35541-35.685) months, 10 cases died from illness in the control group, with a survival rate of 75% and an average survival time of 34.70 (95% CI: 34.559-34.832) months, the survival rate of the observation group was higher than that of the control group, the difference was statistically significant (P<0.05). CONCLUSION:Sequential Auto-HSCT with RVD regimen in the treatment of MM can improve the short-term efficacy and increase the survival rate of patients, which will not increase toxic and side effects and has high safety.

Background: To study the effect of immunophenotypic polymorphisms from donor origin on the recipient minimal residual disease (MRD) detection by flow cytometry, we reported two cases who had cells with aberrant expressions from donor origin after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in bone marrow specimens. Methods: 8-color flow cytometry detected bone marrow MRD according to our conventional panel. Two cases were all recipients after allo-HSCT with complete remission (CR) of morphology and genetics, and 100% donor genotype of chimerism studies. One was acute myeloid leukemia (AML) developing from myelodysplastic/myeloproliferative neoplasms (MDS/MPN, whose bone marrow MRD detection was abnormal for lack of CD16 expression on neutrophils after allo-HSCT. Another was T-lymphoblastic leukemia (T-ALL) diagnosed in other hospital. There were more and more obvious NK cells with abnormal expression in his bone marrow in 10 months after allo-HSCT. Results: Identical phenotypes were recognized in peripheral blood samples obtained subsequently from the respective donors. The neutrophils from donor of MDS/MPN related AML patient lacked CD16 expression, most likely a previously described Fcγ receptor IIIB (FcγRIIIB) gene deletion. The NK cells from donor of T-ALL had abnormal expressions of overwhelmingly CD159c and CD2 deletion which had rarely been reported before. Two donors were all healthy people. Conclusion These donor-specific phenotypic polymorphisms could have an effect on flow cytometric MRD detection of recipient after allo-HSCT. These might be misdiagnosed as MRD positive or relapse if the analyzer lacked experience. It might be a promising method to avoid misdiagnosis that had flow cytometric MRD detection on donor sample using the same panel as recipients.Figure 2Case 2. The immunophenotyping of the recipient BM in 10 months after allo-SCT. The NK cells had aberrant expressions but reactive cells couldn't be excluded.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Case 2. The immunophenotyping of his donor PB. The NK cells (events showed in red color) had the same aberrant expressions as those of the recipient.View Large Image Figure ViewerDownload Hi-res image Download (PPT)