This case report discusses the management of anti-neutrophil cytoplasmic antibodies (ANCA)-negative rapid progressive glomerulonephritis (RPGN) in a 68-year-old man with a complex medical history, presenting with fatigue, edema, and acute renal failure. Despite the absence of positive biomarkers for specific RPGN types, the clinical progression suggested microscopic polyangiitis, leading to intensive immunosuppressive therapy with cyclophosphamide and rituximab. The patient\'s condition was further complicated by the coexistence of nephritic and nephrotic syndromes, requiring nuanced management strategies, including prolonged hemodialysis. After initial treatment failure, remission was eventually achieved, allowing cessation of dialysis and significant recovery of renal function. This case highlights the challenges of diagnosing and managing ANCA-negative RPGN, particularly the importance of a tailored, dynamic approach to treatment in resource-limited settings. The recovery observed underscores the potential for renal function improvement even after prolonged periods of intensive therapy, reinforcing the need for persistence and adaptability in managing complex RPGN cases.

BACKGROUND: Waldenström\'s macroglobulinemia (WM) is defined as a lymphoplasmacytic lymphoma (LPL) involving the bone marrow (BM) with presence of IgM monoclonal protein, and comprises > 95% of all LPL cases. Rituximab-based regimens have been predominant in the management of WM. Infusion-related reactions (IRRs) are a primary concern with rituximab, although it is generally better tolerated with less toxicity than conventional anticancer agents. Here, we present an autopsy case of an elderly man who died suddenly after receiving the initial infusion of rituximab for WM/LPL.
METHODS: An 84-year-old man was found dead in his bedroom. He had undergone the initial intravenous rituximab infusion for progressive anemia related to Waldenström\'s macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) approximately 15 h before death. Although the protocol for rituximab administration and additional medication was considered appropriate, he exhibited several symptoms consistent with infusion-related reactions (IRRs) during the infusion. Autopsy revealed monotonous proliferation of small-to-medium-sized lymphocytic cells in the bone marrow, consistent with the premortem diagnosis of WM/LPL. Additionally, immunoglobulin λ-light chain-derived amyloid (ALλ) deposition was identified in all organs other than the brain. Although ALλ deposition and LPL infiltration were found in the heart, they were not severe enough to cause severe functional impairment. Severe congestion and/or edema were observed in the lungs, liver, and brain. Although significant inflammatory cell infiltration was not found in any organs, laboratory tests revealed elevated serum levels of inflammatory cytokines, including interleukin-1β, interleukin-6, tumor necrosis factor-α and the presence of IgM-λ monoclonal protein.
CONCLUSIONS: Acute IRRs associated with the initial rituximab infusion were the major contributing factor to his sudden unexpected death. The autopsy findings of present case suggest the necessity for thorough monitoring of older patients with WM/LPL undergoing rituximab treatment, particularly when pronounced IRRs occur during the first administration, in addition to investigating complications of WM/LPL before infusion.

UNASSIGNED: In recent years, thanks to significant advances in basic science and biotechnologies, nephrology has witnessed a deeper understanding of the mechanisms leading to various conditions associated with or causing kidney disease, opening new perspectives for developing specific treatments. These new possibilities have brought increased challenges to physicians, who face with a new complexity in disease characterization and selection the right treatment for individual patients.
UNASSIGNED: We chose four therapeutic situations: anemia in chronic kidney disease (CKD), heart failure in CKD, IgA nephropathy (IgAN) and membranous nephropathy (MN). The literature search was made through PubMed.
UNASSIGNED: Anaemia management remains challenging in CKD; a personalized therapeutic approach is often needed. Identifying patients who could benefit from a specific therapy is also an important goal for patients with CKD and heart failure with reduced ejection fraction. Several new treatments are under clinical development for IgAN; interestingly, they target specifically the pathogenetic mechanisms of the disease. The understanding of MN pathogenesis as an autoimmune disease and the discovery of several autoantibodies allows a better characterization of patients. High-sensible techniques for lymphocyte counting open the possibility of more personalized use of anti CD20 therapies.

Membranous nephropathy is the most common cause of nephrotic syndrome (NS) in non-diabetic adults; in 80% of patients it is idiopathic (PMN). PMN has an autoimmune pathogenesis, 70%-85% of patients have increased titer of antibodies to the podocyte membrane antigen PLA2R. The etiological, prognostic and predictive role of the Ab anti-PLA2R is demonstrated. Standard therapy consists in anti-CD20 monoclonal antibody rituximab (RTX) combined with steroids or immunosuppressants according to the risk of progressive loss of kidney function. The immunosuppressive therapies are potentially associated to severe adverse events that lead to protocol suspension. Given their pivotal pathogenetic role, serum clearance of anti-PLA2R with plasmapheresis could have a beneficial impact on NS, particularly in patients not requiring or tolerating standard therapies. In this series, we present three cases of PMN anti-PLA2R related treated with a RTX plus plasmapheresis approach and demonstrate its overall effective role on anti-PLA2R titer and clinical outcomes.

Takayasu\'s arteritis (TAK) manifests as an insidiously progressive and debilitating form of granulomatous inflammation including the aorta and its major branches. The precise etiology of TAK remains elusive, with current understanding suggesting an autoimmune origin primarily driven by T cells. Notably, a growing body of evidence bears testimony to the widespread effects of B cells on disease pathogenesis and progression. Distinct alterations in peripheral B cell subsets have been described in individuals with TAK. Advancements in technology have facilitated the identification of novel autoantibodies in TAK. Moreover, emerging data suggest that dysregulated signaling cascades downstream of B cell receptor families, including interactions with innate pattern recognition receptors such as toll-like receptors, as well as co-stimulatory molecules like CD40, CD80 and CD86, may result in the selection and proliferation of autoreactive B cell clones in TAK. Additionally, ectopic lymphoid neogenesis within the aortic wall of TAK patients exhibits functional characteristics. In recent decades, therapeutic interventions targeting B cells, notably utilizing the anti-CD20 monoclonal antibody rituximab, have demonstrated efficacy in TAK. Despite the importance of the humoral immune response, a systematic understanding of how autoreactive B cells contribute to the pathogenic process is still lacking. This review provides a comprehensive overview of the biological significance of B cell-mediated autoimmunity in TAK pathogenesis, as well as insights into therapeutic strategies targeting the humoral response. Furthermore, it examines the roles of T-helper and T follicular helper cells in humoral immunity and their potential contributions to disease mechanisms. We believe that further identification of the pathogenic role of autoimmune B cells and the underlying regulation system will lead to deeper personalized management of TAK patients. We believe that further elucidation of the pathogenic role of autoimmune B cells and the underlying regulatory mechanisms holds promise for the development of personalized approaches to managing TAK patients.

OBJECTIVE: To compare the prognostic value of two predictive models based on C-reactive protein (CRP) and albumin (ALB), namely the CRP to ALB ratio (CAR) and the Glasgow prognostic score (GPS), in newly diagnosed patients with diffuse large B-cell lymphoma (DLBCL).
METHODS: The data of newly diagnosed DLBCL patients admitted to our center from May 2014 to January 2022 were reviewed. A total of 111 patients who completed at least 4 cycles of R-CHOP or R-CHOP-like chemotherapy with detailed clinical, laboratory data and follow-up information were included. The receiver operating characteristic (ROC) curve was performed to evaluate the predictive value of pre-treatment CAR on disease progression and survival. Furthermore, the association between CAR and baseline clinical, laboratory characteristics of patients was evaluated, and progression-free survival (PFS) and overall survival (OS) were compared between different CAR and GPS subgroups. Finally, the univariate and multivariate COX propor-tional hazard regression models were used to analyze the factors affecting disease outcomes.
RESULTS: ROC curve showed that the area under the curve (AUC) of CAR predicting PFS and OS in DLBCL patients was 0.687 (P =0.002) and 0.695 (P =0.005), respectively, with the optimal cut-off value of 0.11 for both predicting PFS and OS. Compared with the lower CAR (<0.11) group, the higher CAR (≥0.11) group had more clinical risk factors, including age >60 years (P =0.025), ECOG score ≥2 (P =0.004), Lugano stage III-IV (P < 0.001), non-germinal center B-cell-like (non-GCB) subtype (P =0.035), elevated lactate dehydrogenase (LDH) ( P < 0.001), extranodal involved site >1 (P =0.004) and IPI score >2 (P < 0.001). The interim response evaluation of patients showed that the overall response rate (ORR) and complete response rate (CRR) in the lower CAR group were both significantly better than those in the higher CAR group (ORR: 96.9% vs 80.0%, P =0.035; CRR: 63.6% vs 32.5%, P =0.008). With a median follow-up of 24 months, patients with lower CAR had significantly longer median PFS and OS than those with higher CAR (median PFS: not reached vs 67 months, P =0.0026; median OS: not reached vs 67 months, P =0.002), while there was no statistical difference in PFS (P =0.11) and OS (P =0.11) in patients with GPS of 0, 1, and 2. Multivariate Cox regression analysis indicated that only sex (male) and IPI score >2 were independent risk factors for both PFS and OS.
CONCLUSIONS: CAR is significantly correlated with disease progression and survival in DLBCL patients; And compared with GPS, CAR has more advantages in predicting disease outcomes in DLBCL patients.
UNASSIGNED: 比较C反应蛋白与白蛋白比值和格拉斯哥预后评分对弥漫大B细胞淋巴瘤患者预后判断的价值.
UNASSIGNED: 探讨并比较基于C反应蛋白（CRP）和白蛋白（ALB）的两种预测模型——CRP与ALB比值（CAR）和格拉斯哥预后评分（GPS）——在新诊断弥漫大B细胞淋巴瘤（DLBCL）患者预后评估中的价值。.
UNASSIGNED: 回顾性分析本院2014年5月至2022年1月收治的初诊DLBCL患者的资料，纳入至少完成4个周期R-CHOP或R-CHOP样方案化疗，且临床、实验室检查数据以及随访资料均完整的111例患者。根据患者治疗前CAR及随访截止时的生存状态绘制受试者工作特征曲线（ROC），初步判断CAR对疾病进展和生存结局的预测价值，进一步分析CAR与患者基线临床、实验室特征的关系，并比较不同CAR、GPS分组患者的无进展生存期（PFS）和总生存期（OS），采用单因素和多因素COX风险比例回归模型分析影响疾病预后的因素。.
UNASSIGNED: ROC曲线分析结果显示，CAR预测DLBCL患者PFS、OS的曲线下面积（AUC）分别为0.687（P =0.002），0.695（P =0.005），最佳截断值均为0.11；相较于低CAR（<0.11）组，高CAR（≥0.11）组患者具有更多的临床高危因素，包括年龄>60岁（P =0.025）、ECOG评分≥2分（P =0.004）、Lugano分期III-IV期（ P < 0.001）、non-GCB亚型（P =0.035）、乳酸脱氢酶升高（ P < 0.001）、结外病变数>1处（P =0.004）及IPI评分>2分（ P < 0.001）。对患者进行中期疗效评估，低CAR组患者的总有效率（ORR）及完全缓解率（CRR）均明显优于高CAR组（ORR: 96.9% vs 80.0%, P =0.035；CRR: 63.6% vs 32.5%, P =0.008）。中位随访24个月，低CAR组患者的中位PFS及OS均明显优于高CAR组（中位PFS：未达到 vs 67个月，P =0.0026；中位OS：未达到 vs 67个月，P ＝0.002），而GPS 0分、1分和2分3组患者的PFS（P =0.11）和OS（P =0.11）差异均不具有统计学意义。多因素分析显示，仅有性别为男性和IPI评分>2分是影响患者PFS和OS的独立危险因素。.
UNASSIGNED: CAR与DLBCL患者的疾病进展和生存显著相关；与GPS相比，CAR对患者的预后判断价值更大。.

OBJECTIVE: To investigate the clinical efficacy and prognosis of Rituximab combined with DHAX and CHOP regimen in the first-line treatment of elderly patients with newly diagnosed diffuse large B-cell lymphoma (DLBCL).
METHODS: A total of 36 elderly patients with DLBCL who were admitted and treated with 3 of more courses of treatment from August 2011 to August 2021 were retrospectively analyzed, and they were divided into rituximab±DHAX (R±DHAX) regimen group (18 cases) and rituximab±CHOP (R-CHOP) regimen group (18 cases) according to the treatment plan, and clinical features, efficacy and survival of the patients were observed.
RESULTS: Compared with R-CHOP group, patients of the R±DHAX group were older, and had worse performance status and higher IPI score, the differences between two groups in age, ECOG score and IPI score were statistically significant ( P =0.005 P =0.018, P =0.035), but there were no significant differences beween two groups in gender, whether there were B symptoms, whether LDH was elevated, whether there was extranodal involvement, cell origin, bone marrow infiltration, and whether rituximab was combined ( P =0.738, P =1, P =0.315, P =0.305, P =0.413, P =0.177, P =0.711, P =0.229). The efficacy could be evaluated in 36 cases, including CR 14 (38.9%), PR 17 (47.2%), PD 5 (13.9%), and ORR of 86.1% (31/36). There were no statistically significant differences in CR［(27.8%（5/18） vs 50.0%（9/18）; P >0.05］ and PR ［44.4%（8/18） vs 50.0%（9/18）; P >0.05］ of R±DHAX group and R-CHOP group, there was statistically significant difference in ORR［72.2%（13/18） vs 100.0%（18/18）; P =0.045］ between two groups. The 1-year OS of R±DHAX group and R-CHOP group was (38.9±11.5%)% and (94.4±7.4%)%, respectively, 2-year OS was (16.7±8.8)% and (72.2±10.6)%, respectively, and the differences between two groups were statistically significant ( P =0.001, P =0.002). The median survival time in the R±DHAX group was 11 months(95%CI :8.9-13.1), and the median survival time in the R-CHOP group was not reached, and there was a statistically significant difference between the groups (P < 0.001).
CONCLUSIONS: For elderly DLBCL patients, R±DHAX may not be superior to R-CHOP in OS, and ECOG score, IPI score and age may affect the survival of elderly DLBCL patients. However, R±DHAX regimen is safe, tolerable and has a certain efficacy, which can be used as one of the clinical treatment options for elderly DLBCL.
UNASSIGNED: 利妥昔单抗联合DHAX方案与CHOP方案一线治疗老年初诊弥漫大B细胞淋巴瘤的回顾性研究.
UNASSIGNED: 探讨R±DHAX方案与R-CHOP方案一线治疗老年初诊弥漫大B细胞淋巴瘤（DLBCL）患者的临床疗效及预后。.
UNASSIGNED: 回顾性分析江苏大学附属医院血液科2011年8月—2021年8月收治并至少完成3疗程的36例老年DLBCL患者，根据治疗方案分为利妥昔单抗±DHAX（R±DHAX）方案组（18例）和利妥昔单抗联合CHOP（R-CHOP）方案组（18例），观察患者的临床特征、疗效及生存。.
UNASSIGNED: 与R-CHOP组比较，R±DHAX组患者更高龄、体能状态更差及IPI评分更高，在年龄、ECOG评分、IPI评分上两组间差异有统计学意义(P =0.005，P =0.018，P =0.035)，而在性别、有无B症状、LDH是否升高、有无结外累及、细胞来源、有无骨髓浸润、是否联合使用利妥昔单抗上两组间差异均无统计学意义(P =0.738，P =1，P =0.315，P =0.305，P =0.413，P =0.177，P =0.711，P =0.229)。36例患者均可评价疗效，其中CR 14例（38.9%）、PR 17例（47.2%）、PD 5例（13.9%），ORR为86.1%（31/36）。R±DHAX组和R-CHOP组CR［(27.8%（5/18）对50.0%（9/18）］、PR［44.4%（8/18）对50.0%（9/18）］差异无统计学意义，但ORR［72.2%（13/18）对100.0%（18/18）］差异有统计学意义(P =0.045)。R±DHAX组和R-CHOP组1年OS率分别为（38.9±11.5）%和（94.4±7.4）%，2年OS率分别为（16.7±8.8）%和（72.2±10.6）%，组间比较差异有统计学意义（P =0.001，P =0.002）。R±DHAX组中位生存时间11个月（95%CI ：8.9-13.1），R-CHOP组中位生存时间未达到，组间存在统计学差异(P <0.001)。.
UNASSIGNED: 对于老年DLBCL患者，在OS方面R±DHAX方案可能不优于R-CHOP方案，且ECOG评分、IPI评分、年龄可能影响老年DLBCL患者生存。但R±DHAX方案安全、可耐受且具有一定疗效，可作为临床上老年DLBCL治疗选择之一。.

OBJECTIVE: To investigate the effectiveness, safety, and related prognostic factors of the treatment of follicular lymphoma (FL) with a regimen containing Bendamustine.
METHODS: The clinical data of 129 FL patients who were treated with Bendamustine containing regimen were collected from January 1,2020 to October 30,2022 in the Hematology Department of Lianyungang Second People\'s Hospital and Jiangsu Provincial People\'s Hospital. The patients were divided into three groups: Bendamustine plus Rituximab (BR), Bendamustine plus Obinutuzumab (GB), Rituximab + Cyclophosphamide + Epirubicin / Doxorubicin + Vindesine + Prednisone (R-CHOP). The efficacy, safety and related prognostic factors of the treatment of FL with a regimen based on Bendamustine were retrospectively analyzed.
RESULTS: The ORR was 98% for the BR group, 94% for the GB group, and 72.3% for the R-CHOP group, while the CR rate was 61.2%,70% and 40.4%, respectively. The ORR and CR rates of the R-CHOP group were statistically different from those of the BR group and GB group (P < 0.05). The 3-year PFS rate of the BR group, GB group, and R-CHOP group was 89.6%, 90.9%, 48.9%, respectively. There was a statistically significant difference in 3-year PFS between the R-CHOP group, BR group, and GB group (P < 0.05), while there was no statistically significant difference in 3-year OS（P ＞0.05）. Hematological adverse reactions were mainly bone marrow suppression. Lymphocytes and CD4+T lymphocytes decreased to the lowest level about 6 months after treatment, and the incidence of lymphopenia in BR group and GB group was higher than that in R-CHOP group, with a statistical difference (P < 0.05). The higher incidence of non-Hematological adverse reactions were pulmonary infection, EB virus infection, hepatitis B virus reactivation, and gastrointestinal reactions without statistical difference in 3 groups (P >0.05), and were all controllable. The Receiver operating characteristic of CD4+T lymphocyte count showed that AUC of BR group was 0.802, and the critical value was 258/uL; AUC of GB group was 0.754 with a critical value of 322/uL.
CONCLUSIONS: The treatment of FL with the Bendamustine containing regimen has good efficacy and controllable adverse reactions, but lymphocytopenia was significant after treatment, and the curative efficacy in combination with various CD20 monoclonal antibodies was different. The lowest CD4+T lymphocyte count can be used as a predictive factor for the occurrence of infection and efficacy of the Bendamustine containing regimen for FL.
UNASSIGNED: 苯达莫司汀治疗滤泡性淋巴瘤后CD4+T淋巴细胞减少或可预测感染发生及疗效.
UNASSIGNED: 探讨含苯达莫司汀方案治疗滤泡性淋巴瘤（FL）的有效性、安全性及相关预后因素。.
UNASSIGNED: 收集2020年１月１日-2022年10月30日在连云港市第二人民医院与江苏省人民医院血液科应用含苯达莫司汀方案治疗的129例FL患者的临床资料。将患者分为苯达莫司汀联合利妥昔单抗（BR）组、苯达莫司汀联合奥妥珠单抗（GB）组、利妥昔单抗+环磷酰胺+表柔比星/多柔比星+长春地辛+泼尼松（R-CHOP）组，回顾性分析苯达莫司汀为基础的方案治疗FL的疗效、安全性及相关预后因素。.
UNASSIGNED: BR组、GB组与R-CHOP组的ORR分别为98%、94%、72.3%，CR率分别为61.2%、70%、40.4%，R-CHOP组的ORR率、CR率与BR组、GB组均具有统计学差异（P < 0.05）。BR组、GB组与R-CHOP组的3年PFS率分别为89.6%、90.9%、48.9%，具有统计学差异（P < 0.05），但3年OS无统计学差异（P ＞0.05）。血液学不良反应主要为骨髓抑制，淋巴细胞及CD4+T淋巴细胞在用药后6个月降至最低，且BR组与GB组淋巴细胞减少的发生率高于R-CHOP组，具有统计学差异（P < 0.05）；非血液学不良反应发生率较高的依次为肺部感染、EB病毒感染、乙肝病毒再激活、胃肠道反应，均可控，组间比较无统计学差异（P ＞0.05）。CD4+T淋巴细胞计数ROC曲线示，BR组判断感染的AUC为0.802，临界值为258/uL；GB组判断感染的AUC为0.754，临界值为322/uL。.
UNASSIGNED: 含苯达莫司汀方案治疗FL疗效佳，且不良反应可控，但治疗后淋巴细胞减少明显且与不同CD20单抗联合使用时表现出差异性。CD4+T淋巴细胞计数最低值可以作为含苯达莫司汀方案治疗FL感染发生及疗效的预测因素。.

Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration.
CONCLUSIONS: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests.
BACKGROUND: • During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring.
BACKGROUND: • There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment. • This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests.

Autoimmune haemolytic anaemia (AIHA), autoimmune destruction of erythrocytes is most commonly secondary to immunomodulated conditions. The association between AIHA and inflammatory bowel disease (IBD) has been poorly investigated. We aim to report a case of AIHA in a patient with ulcerative colitis (UC) treated with vedolizumab.A case of a woman in her 30s with UC that after the initiation of vedolizumab developed severe anaemia. Due to the absence of visible blood losses and a positive Coombs direct test, the diagnosis of AIHA was established. The patient initially initiated prednisolone with no response. Rituximab had to be introduced. After a few days with this therapy, there was a clinical and analytical improvement.AIHA must be taken into account as a possible cause of anaemia in patients with IBD. The differential diagnosis between IBD or drug-related (namely vedolizumab) as the cause of the AIHA is complex and almost impossible to establish.