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The optimal method of tumor burden evaluation in newly diagnosed multiple myeloma (NDMM) is yet to be determined. This study aimed to compare the value of 11 C-acetate positron-emission tomography (PET)/computed tomography (CT) (AC-PET and 18 F-fluorodeoxyglucose PET/CT (FDG-PET) in the assessment of tumor burden in NDMM. This study evaluated 64 NDMM patients between February 2015 and July 2018. AC-PET and FDG-PET were used to assess myeloma lesions. The clinical data, imaging results, and their correlations were analyzed. Diffuse bone marrow uptake in AC-PET was significantly correlated with biomarkers for tumor burden, including serum hemoglobin ( P  = 0.020), M protein ( P  = 0.054), the percentage of bone marrow plasma cells ( P  < 0.001), and the Durie–Salmon stage of the disease ( P  = 0.007). The maximum standard uptake value (SUV max ) of focal lesions and high diffuse bone marrow uptake in AC-PET showed stronger correlations with high-risk disease ( P  = 0.017, P  = 0.013) than those in FDG-PET. Moreover, the presence of diffuse bone marrow uptake, more than ten focal lesions, and an SUV max of focal lesions of > 6.0 in AC-PET, but not in FDG-PET, predicted a higher probability of disease progression and shorter progression-free survival ( P  < 0.05). AC-PET outperformed FDG-PET in tumor burden evaluation and disease progression prediction in NDMM.

Background Bruton tyrosine kinase (BTK) inhibitors have demonstrated a high degree of efficacy in the treatment of B cell malignancies characterized by constitutive B cell receptor activation, including chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Methods The efficacy and safety of zanubrutinib, an investigational highly selective BTK inhibitor, was evaluated in this single-arm, phase 2 study of Chinese patients with relapsed/refractory CLL/SLL. The primary endpoint was overall response rate as assessed by an independent review committee. Results Of the 91 evaluable patients, 77 (84.6%) achieved a response, with three (3.3%), 54 (59.3%), and 20 (22%) patients achieving a complete response, partial response, and partial response with lymphocytosis, respectively, after a median follow-up of 15.1 months. The estimated 12-month event-free rate for duration of response was 92.9%. The most commonly reported grade ≥ 3 adverse events (AEs) were neutropenia (44%), thrombocytopenia (15.4%), lung infection/pneumonia (13.2%), upper respiratory tract infection (9.9%), and anemia (8.8%). The 12-month overall survival rate was 96%. Eight (9.0%) patients discontinued zanubrutinib due to AEs, and seven (8.0%) patients required at least one dose reduction. Conclusion Treatment of patients with relapsed/refractory CLL/SLL with zanubrutinib was generally well tolerated and resulted in a high overall response rate, thereby conferring a favorable benefit-risk profile. Trial registration Prospectively registered in China public registry (CTR20160890) on December 7, 2016: http://www.chinadrugtrials.org.cn/ . Retrospectively registered in ClinicalTrials.gov ( NCT03206918 ) on July 2, 2017.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is widely used to treat malignant hematological neoplasms and non-malignant hematological disorders. Approximately, 5000 allo-HSCT procedures are performed in China annually. Substantial progress has been made in haploidentical HSCT (HID-HSCT), pre-transplantation risk stratification, and donor selection in allo-HSCT, especially after the establishment of the “Beijing Protocol” HID-HSCT system. Transplant indications for selected subgroups in low-risk leukemia or severe aplastic anemia (SAA) differ from those in the Western world. These unique systems developed by Chinese doctors may inspire the refining of global clinical practice. We reviewed the efficacy of allo-HSCT practice from available Chinese studies on behalf of the HSCT workgroup of the Chinese Society of Hematology, Chinese Medical Association and compared these studies to the consensus or guideline outside China. We summarized the consensus on routine practices of all-HSCT in China and focused on the recommendations of indications, conditioning regimen, and donor selection.

Background Intravascular large B-cell lymphoma (IVLBCL) is an ultra-orphan disease (incidence 0.095/million/year) with dismal prognosis due to delayed diagnosis and limited therapeutic options. We aimed to evaluate a novel diagnostic algorithm and the efficacy of zanubrutinib-containing therapy in a real-world Chinese cohort. Methods This single-center retrospective study enrolled 54 IVLBCL patients (2010–2022). Diagnostic approaches evolved from PET/CT-guided biopsy to a combination of serum interleukin-10 (IL-10, cut-off 95.65 pg/mL), random skin biopsy (RSB), and circulating tumor DNA (ctDNA) profiling. Treatment regimens shifted from R-CHOP to methotrexate-based therapy (MTX), then to zanubrutinib plus R-CHOP (ZR-CHOP). Results The cohort exhibited distinct features: 35.2% Asian-variant IVLBCL, 50% CNS involvement, and 22.9% PET/CT negativity. IL-10 combined with RSB enabled the diagnosis of 11 PET/CT-negative patients who would have otherwise remained undiagnosed. ctDNA revealed MYD88 L265P (11/17, 65%) and CD79B (7/17, 41%) variants. ZR-CHOP ( n  = 22) achieved significantly superior 2-year progression-free survival (PFS) compared to R-CHOP alone ( n  = 6) (90% vs. 30%; hazard ratio [HR] 0.031, P  < 0.0001) and demonstrated 100% central nervous system (CNS) relapse-free survival. The efficacy of ZR-CHOP was comparable to that of methotrexate (MTX)-based therapy ( n  = 20; 2-year PFS 85%), despite a shorter median follow-up (20.1 vs. 38.0 months). Conclusions In this largest Asian IVLBCL cohort to date, IL-10 + RSB + ctDNA significantly improved diagnostic accuracy. Zanubrutinib demonstrated promising efficacy, particularly in CNS involvement, offering a pragmatic solution for this orphan disease.

Although CAR-T cell therapy has emerged as a promising therapeutic option for relapsed/refractory (R/R) diffuse large B-cell lymphoma (DLBCL), long manufacturing time, high cost and toxicities limit its clinical use. T-cell engagers (TCEs) including glofitamab may address these unmet needs, but real-world data remain scarce. This real-world study (NCT06481826) enrolled 30 Chinese patients. The median prior treatment lines were 2 (2–5) and 90% had no prior CAR-T therapy. After a median follow-up of 15 months, the overall response rate (ORR) was 66.7%. The 1-year progression-free survival (PFS) was 62.5% with a median PFS of 15 months. The 1-year overall survival (OS) was 70%. Univariate analysis identified more than 1 extranodal involvement site, TP53 mutation and low baseline lymphocytes as poor prognostic factors. Safety profile was manageable. 43.3% patients developed cytokine release syndrome (CRS, mostly grade 1–3) and 3.3% had grade 1 immune effector cell-associated neurotoxicity syndrome (ICANS). The hematologic toxicity was mostly mild. COVID-19 infection occurred in 20% of patients. This study demonstrates that glofitamab is effective and well-tolerated in Chinese R/R DLBCL patients. Earlier lines of glofitamab may be more beneficial, while expanded cohorts are warranted to explore its efficacy among patients with multiple extranodal lesions, TP53 mutations and low baseline lymphocytes. Highlights Glofitamab is effective and well-tolerated in Chinese R/R DLBCL patients. Multiple extranodal lesions, TP53 mutations and low baseline lymphocytes are associated with poor response to glofitamab. Earlier lines of glofitamab may be more beneficial.

Evans syndrome, characterized by autoimmune hemolytic anemia and immune thrombocytopenia, is rare and often refractory to conventional therapies. In Chinese patients with systemic lupus erythematosus (SLE), its incidence is approximately 0.47%. We report the first case of anti-CD19 chimeric antigen receptor (CAR) T cell therapy for SLE-Evans syndrome. A 36-year-old woman with refractory disease received autologous CD19 CAR T cells (inati-cel) after lymphodepleting chemotherapy. CAR T cells expanded rapidly, peaking on day 7, and remained detectable for 6 months. CD19 B cells were eliminated by day 14. Hemoglobin levels improved from 48 g/L pre-infusion to 115 g/L at day 28 and stabilized. Platelet counts increased from 60,000/µL to 195,000/µL at 2 months. Only grade 1 cytokine release syndrome occurred, with no other adverse events. This case demonstrates that CD19 CAR T cell therapy is a promising approach for refractory Evans syndrome, warranting further investigation.

Early diagnosis of primary central nervous system lymphoma (PCNSL) represents a challenge, and cerebrospinal fluid (CSF) cytokines may be diagnostic biomarkers for PCNSL. We used an electrochemiluminescence immunoassay to measure interleukin (IL)-10, IL-6, IL-8 and tumor necrosis factor α (TNF-α) in the CSF of 22 B cell PCNSL patients and 80 patients with other CNS diseases. CSF IL-10 was significantly higher in PCNSL patients than in the control group (median 74.7 pg/ml vs < 5.0 pg/ml, P  < 0.000). Using a CSF IL-10 cutoff value of 8.2 pg/ml, the diagnostic sensitivity and specificity were 95.5% and 96.1%, respectively (AUC, 0.957; 95% CI, 0.901–1.000). For a CSF IL-10/IL-6 cutoff value of 0.72, the sensitivity was 95.5%, and the specificity was 100.0% (AUC, 0.976; 95% CI, 0.929–1.000). An increased CSF IL-10 level at diagnosis and post-treatment was associated with poor Progression free survival (PFS) for patients with PCNSL ( P  = 0.0181 and P  = 0.0002, respectively). A low diagnostic value for PCNSL was found with CSF IL-8 or TNF-α. In conclusion, increased CSF IL-10 was a reliable diagnostic biomarker for large B cell PCNSL, and an IL-10/IL-6 ratio facilitates differentiation from other conditions, especially a CNS infection.