Castleman disease (CD), a rare and clinically heterogeneous condition, frequently involves renal impairment, though this relationship remains poorly characterized. This large cohort study of 183 patients (116 unicentric [UCD], 67 multicentric [MCD]) investigated renal involvement (RI). RI occurred in 6.03

Background : Tumors co-occurring with chronic kidney disease and multiple infections present a rare and clinically challenging scenario. Case presentation : A 62-year-old man presented edema with moderate kidney impairment and a refractory rash. Diagnostic workup revealed atypical membranous nephropathy, positive antibodies for hepatitis C virus and liver fluke, and hypereosinophilia. Renal biopsy confirmed membranous nephropathy, while lymph node biopsy identified classical Hodgkin lymphoma of mixed cellularity type, with detectable Epstein–Barr virus DNA and associated bone marrow eosinophilia. Treatment with anti-pathogen agents and medium-dose corticosteroids resulted in symptomatic and laboratory improvement, following which the patient was transitioned to oncology care. To our knowledge, this is the first reported case of concurrent mixed cellularity Hodgkin lymphoma, atypical membranous nephropathy, hepatitis C virus infection, and liver fluke seropositivity. Conclusion : This unique presentation provides clinical insights that may help clarify underlying pathophysiological links and inform management strategies for similar complex cases.

The clinical diagnosis and treatment processes of four children with Castleman disease admitted to Guangzhou Women and Children's Medical Center,Guangzhou Medical University,from March 2021 to December 2024 were retrospectively analyzed.Among them,three cases were unicentric Castleman disease,presenting as abdominal pain with ileocecal ulceration,concomitant classical Hodgkin lymphoma,and choledochal cyst,respectively.One case was severe idiopathic multicentric Castleman disease involving lymph nodes in the neck and axillary regions,presenting with fever,abdominal distension,generalized edema,fatigue,anemia,hepatic dysfunction,polyserous effusion,and other systemic manifestations.The three unicentric cases underwent complete surgical resection of enlarged lymph nodes.The case with abdominal pain received subsequent enteral nutrition and oral thalidomide therapy.The case complicated with Hodgkin lymphoma received chemotherapy according to the Hodgkin lymphoma protocol.The severe multicentric case was treated with rituximab combined with vincristine,pirarubicin,cyclophosphamide,and prednisone.All four cases achieved complete remission during 6 to 30 months of follow-up.Clinical manifestations of Castleman disease in children lack specificity.Surgical resection remains the main treatment for unicentric cases,while treatment regimens for multicentric cases are more varied.The overall prognosis is favorable.Further studies are needed to determine the optimal treatment for Castleman disease complicated by gastrointestinal ulcers and severe idiopathic multicentric Castleman disease.

Secondary hemophagocytic lymphohistiocytosis (HLH) syndrome, a fatal disorder characterized by NK/T-cell deficiency, cytokine storm, and organ damage, is rare in chronic lymphocytic leukemia (CLL). Ibrutinib, the first generation of irreversible Bruton's tyrosine kinase inhibitor, has been the first-line therapy for CLL. As an off-target effect, it can also block IL-2 inducible T-cell kinase (ITK), which is essential in maintaining normal NK and T-cell functions. Up to now, 4 cases reported secondary HLH developed in CLL patients shortly after ibrutinib therapy, which indicated ibrutinib might be associated with HLH via NK/T cell damage as a result of ITK inhibition. We herein report the first case describing EBV-driven HLH developed in a CLL patient under long-term ibrutinib monotherapy (4 year), also showing concurrent NK and T cell deficiency. Therefore, the relationship between the long-term use of ibrutinib and the pathophysiology of HLH, as well as the mediating role of NK/T cell disorder caused by ITK blockade therein, deserves attention and further studies.

EBV-positive diffuse large B-cell lymphoma (EBV + DLBCL) is associated with poor prognosis, possibly due to the capacity of EBV to dampen host anti-tumor immunity. Patients’ peripheral EBV antigen-specific T lymphocytes may be functional deficiency. This study investigated the mechanisms underlying this deficiency by examining the phenotypes and function of peripheral T cells via ELISPOT and flow cytometry. 6 EBV + DLBCL patients, 54 EBV-negative DLBCL (EBV- DLBCL) patients, and 12 healthy controls were enrolled. We observed significantly reduced IFN-γ secreting T cells in EBV + patients upon EBV peptides stimulation compared to EBV-negative patients (P < 0.001), indicating a dysfunction in EBV antigen-specific T cells. Furtherly, compared to EBV- DLBCL, EBV + DLBCL showed decreased proportions of total lymphocytes (P = 0.005), CD8+ T cells (P = 0.004), CD4+ T cells central memory (P = 0.017), CD8+ T cells naïve (P = 0.001), and CD8+ T cells effector (P = 0.031), alongside increased CD4+ and CD8+ effector memory T cells (P = 0.010 and P < 0.001, respectively). Both CD4+ and CD8+ T cells demonstrated elevated PD-1 expression (P = 0.045 and P = 0.036, respectively), and the CD4+TIM-3−CTLA-4− population was reduced (P = 0.049), in EBV + DLBCL. There was also a significant decline in CD28+KLRG1− (P = 0.018) and CD28+CD57−KLRG1− (P = 0.036) subsets among CD8+ T cells in EBV + patients. CD8+ T cells showed decreased IFN-γ expression after PMA/BFA stimulation in EBV + DLBCL(P = 0.015). These findings suggested that EBV antigen-specific T cell functional deficits might correlate with altered T cell subset distributions, heightened levels of exhaustion and senescence, and diminished expression of immune effector molecules.

Background A former cohort study has raised concern regarding the unanticipated hazard of omeprazole in expediting osteoarthritis (OA) advancement. The precise nature of their causal evidence, however, remains undetermined. The present research endeavors to investigate the underlying causal link between omeprazole and OA through the application of mendelian randomization (MR) analysis. Methods The study incorporated the ukb-a-106 and ukb-b-14,486 datasets. The investigation of causal effects employed methodologies such as MR-Egger, Weighted median, Inverse variance weighted (IVW) with multiplicative random effects, and IVW (fixed effects). The IVW approach was predominantly considered for result interpretation. Sensitivity analysis was conducted, encompassing assessments for heterogeneity, horizontal pleiotropy, and the Leave-one-out techniques. Results The outcomes of the MR analysis indicated a causal relationship between omeprazole and OA, with omeprazole identified as a contributing risk factor for OA development (IVW model: OR = 1.2473, P < 0.01 in ukb-a-106; OR = 1.1288, P < 0.05 in ukb-b-14,486). The sensitivity analysis underscored the robustness and dependability of the above-mentioned analytical findings. Conclusion This study, employing MR, reveals that omeprazole, as an exposure factor, elevates the risk of OA. Considering the drug's efficacy and associated adverse events, clinical practitioners should exercise caution regarding prolonged omeprazole use, particularly in populations with heightened OA risks. Further robust and high-quality research is warranted to validate our findings and guide clinical practice.

Patients with hematological malignancies who experience severe infections are at risk of developing dangerous complications due to excessive inflammatory cytokines. To improve the prognosis, it is crucial to identify better ways to manage the systemic inflammatory storm after infection. In this study, we evaluated four patients with hematological malignancies who developed severe bloodstream infections during the agranulocytosis phase. Despite receiving antibiotics, all four patients presented elevated serum IL-6 levels as well as persistent hypotension or organ injury. Adjuvant therapy with tocilizumab, an IL-6-receptor antibody, was administered, and three of the four patients showed significant improvement. Unfortunately, the fourth patient died due to multiple organ failure caused by antibiotic resistance. Our preliminary experience suggests that tocilizumab, as an adjuvant therapy, may help alleviate systemic inflammation and reduce risk of organ injury in patients with elevated IL-6 levels and severe infection. Further randomized controlled trials are needed to confirm the effectiveness of this IL-6 targeting approach.

Unfit acute myeloid leukemia and high-risk myelodysplastic syndrome patients with prolonged neutropenia demand coadministration of venetoclax and azoles. However, venetoclax dosing under drug-drug interaction with azoles remains controversial. Therapeutic drug monitoring (TDM) is expected to guide drug dosage adjustments. We retrospectively enrolled 17 patients under this coadministration and TDM. Venetoclax dosages were interfered when inappropriate drug concentrations appeared. The primary endpoints were objective response and adverse events. Venetoclax concentration outliers were more frequently evaluated before than after dose adjustment (Cmax 60.87% vs. 0.00%, p < .0001). MRD negativity rate was higher in patients staying within reference range than those having outliers (90.91% vs. 33.33%, p = .028). Objective response rate was 100%. Hematologic adverse events included neutropenia (93.3%), febrile neutropenia (53.3%), and thrombocytopenia (81.3%). Median time to neutropenia and thrombocytopenia recovery was 20 (14-32) and 16.5 (6-34) days, respectively. No invasive fungal and other life-threatening infections were observed.

The purpose is to ascertain the clinical impact of Castleman disease (CD) by reassessment of the real-world data from Peking University First Hospital (PKUFH). The results will contribute to the standardization of diagnosis and treatment on CDs. Based on the last 15-year retrospective real-world data from Peking University First Hospital (PKUFH), we reclassified and re-evaluated the clinical and pathological information of patients with pathologically suspected diagnosis of CD. A total of 203 patients were included in our study, in which the diagnosis of CD was confirmed in 189 cases, including 118 patients with unicentric CD (UCD, n = 118, 62.4%) and 71 patients with multicentric CD (MCD, n = 71, 37.6%). A total of 44.1% (n = 52) of UCDs in our cohort were complicated with paraneoplastic pemphigus (PNP). The treatment of UCD is primarily surgical, with a 5-year overall survival (OS) of 88.1%. Patients with PNP had a poorer prognosis than those without PNP (82.9% (95% CI 123-178) vs 92.8% (95% CI 168-196), log-rank p = 0.041). The rate of concurrent systemic symptoms was 74.6% (n = 53), and renal involvement occurred in 49.3% (n = 35) MCD patients. The MCD treatments were mainly chemotherapy regimens, with a 5-year OS of 77.6% (95% CI, 143-213). Patients with UCD demonstrate a better overall prognosis than patients with MCD. But the prognosis of those complicated with PNP was poor. The differential diagnosis of MCD is extensive. MCD treatment in China is heterogeneous. The inaccessibility of anti-IL-6-targeted drugs in China may contribute to the poor prognosis for patients with MCD.A preprint has previously been published (Guo et al. 34).

2577 Background: Sintilimab plus bevacizumab has been approved as first-line treatment in patients (pts) with advanced hepatocellular carcinoma (HCC). Dual inhibition of PD-1 and lymphocyte-activation gene 3 (LAG-3) may improve anti-tumor effect synergistically. IBI110 plus sintilimab has shown preliminary antitumor activity in advance solid tumors. This phase Ib cohort study evaluated the efficacy, tolerability, and safety of IBI110 in combination with sintilimab and lenvatinib as first-line treatment in pts with advanced HCC. Methods: This phase Ib study enrolled pts with histologically or cytologically diagnosed or clinically confirmed unresectable or metastatic hepatocellular carcinoma, no previous systemic treatment. Patients received IBI110 200mg IV Q3W and sintilimab 200mg IV Q3W, in combination with lenvatinib (weight <60kg: 8mg, weight ≥60 kg: 12mg) orally administered once daily, until disease progression, unacceptable toxicity or death. The primary objective was to evaluate the safety, tolerability, and efficacy of the combination therapy per RECIST v1.1. Results: As the data cutoff date on January 9 th 2023, 28 pts were enrolled (median age: 59 years; ECOG PS=0: 6 pts; stage C per Barcelona Clinic Liver Cancer [BCLC]: 13 pts). All 28 pts were included for safety analysis. Treatment-related adverse events (TRAEs) of any grade occurred in 27 pts (96.4%) and the most frequent TRAEs included hypertension (50%), hypothyroidism (42.9%), neutrophil count decreased (39.3%). Grade ≥ 3 TRAEs occurred in 16 pts (57.1%) and grade ≥3 irAEs occurred in 4 (14.3%) pts. Two (7.1%, 2/28) pts experienced TRAE leading to drug discontinuation. One pt experienced investigator-assessed treatment related death. As of data cutoff, for 27 pts with at least one efficacy evaluation, the objective response rate (ORR) was 29.6% (95% CI, 13.8-50.2). The disease control rate (DCR) was 85.2% (95% CI, 66.3-95.8), and 11 pts were still in treatment. With a median follow up of 12.2 months (95%CI, 11.0-12.6), the median progression-free survival (PFS) was 9.9 months (95%CI, 5.7-NC) and the 9-month PFS rate was 54.5% (95%CI, 33.8-71.2). The median overall survival (OS) was not reached. Conclusions: In this phase Ib study, IBI110 in combination with sintilimab and lenvatinib as first line therapy demonstrated robust response and survival benefit with manageable safety profile in patients with advanced hepatocellular carcinoma. This combination therapy need to be further explored in this population. Clinical trial information: NCT04085185 .

Rapidly progressive interstitial lung disease (RP-ILD) clearly harms the prognoses of dermatomyositis/polymyositis (DM/PM) patients, however there is a dearth of numerical prevalence and therapy comparison in this field. Therefore, the purpose of this study was to determine the prevalence of RP-ILD in DM/PM patients and compare prognoses, including remission rate and survival data, between treatments. Studies with reports of RPILD in DM/PM patients and studies with definite remission and/or survival data of DM/PM-RP-ILD were included in the study. Data sources were Pubmed, Embase, and Cochrane Library without language restrictions. Two authors (WHL and WWQ) extracted independently the data. Estimates of the pooled effects were calculated using the Mantel-Haenszel technique (random effects). The prevalence meta-analysis included 18 papers with 6058 DM/PM patients, and 31 papers were analyzed for treatment effects, including remission rate, 6-month survival rate, 1-year survival rate, and 5-year survival rate. Database search yielded 1816 articles. In the DM/PM population, the combined prevalence of RP-ILD was 8.9% (95% CI, 5.8% to 12.1%). Patients with RP-ILD have a remission rate of 58.4% (95% CI, 47.3% to 69.4%), with biologic treatment with the highest remission rate, followed by triple therapy (defined as adding a third intravenous medication, including cyclophosphamide and immunoglobulin). Biologics therapy had the highest overall survival rate at six months (95% CI, 49.8% to 73.9%), followed by cDMARDs, plasma exchange, and triple therapy. The 1-year survival rate was 77.4% (95% CI, 66.7% to 88.1%), and triple therapy and cDMARDs had the best survival rates. The 5-year survival rate was 40.0% (95% CI, 10.0% to 69.9%). The prevalence of RP-ILD in DM/PM was approximately 8.9%, with a poor long-term prognosis. The use of biological agents appears to provide the best therapeutic outcomes, providing RP-ILD management with a novel evidence-based therapy. The use of strong immunosuppressive treatments may result in life-threatening side effects, thus clinicians must closely monitor the condition.

Background 5-Hydroxymethylcytosine (5hmC), an important DNA epigenetic modification, plays a vital role in tumorigenesis, progression and prognosis in many cancers. Diffuse large B cell lymphoma (DLBCL) can involve almost any organ, but the prognosis of patients with DLBCL at different primary sites varies greatly. Previous studies have shown that 5hmC displays a tissue-specific atlas, but its role in DLBCLs at different primary sites remains unknown. Results We found that primary gastric DLBCL (PG-DLBCL) and lymph node-involved DLBCL (LN-DLBCL) patients had a favorable prognosis, while primary central nervous system DLBCL (PCNS-DLBCL) patients faced the worst prognosis, followed by primary testicular DLBCL (PT-DLBCL) and primary intestinal DLBCL (PI-DLBCL) patients. Thus, we used hmC-CATCH, a bisulfite-free and cost-effective 5hmC detection technology, to first generate the 5hmC profiles from plasma cell-free DNA (cfDNA) of DLBCL patients at these five different primary sites. Specifically, we found robust cancer-associated features that could be used to distinguish healthy individuals from DLBCL patients and distinguish among different primary sites. Through functional enrichment analysis of the differentially 5hmC-enriched genes, almost all DLBCL patients were enriched in tumor-related pathways, and DLBCL patients at different primary sites had unique characteristics. Moreover, 5hmC-based biomarkers can also highly reflect clinical features. Conclusions Collectively, we revealed the primary site differential 5hmC regions of DLBCL at different primary sites. This new strategy may help develop minimally invasive and effective methods to diagnose and determine the primary sites of DLBCL.

The efficacy and safety of Epstein-Barr virus (EBV)-specific antigen peptide-activated cytotoxic T lymphocytes (CTLs) in the treatment of refractory or recurrent angioimmunoblastic T-cell lymphoma (AITL) was determined in this prospective one-arm clinical study. Seven males and two females were enrolled with a median age of 70 years. The tumor stages were all stage III and IV. All patients had group B symptoms and IPI scores of 3 to 5 points. All patients received chemotherapy before CTLs infusion which the median chemotherapy cycle was three. The diseases states before CTLs included five cases of disease progression (PD), two cases of recurrence (R), and two cases with residual lesions after chemotherapy. Eight patients received HLA-haploidentical EBV-specific CTLs, and one patient chose autologous CTLs. The number of transfused cells was 1.67 to 2.38 × 1010 for one course of CTLs therapy. One patient was treated with three courses of CTLs, three patients were treated with two courses of CTLs, and five patients were treated with one course of CTLs. During the infusion, eight patients had fever, one patient had rash, and no graft-vs-host diseases were observed. The EBV-DNA decreased by more than two orders of magnitude in six patients, and the response rate was 66.7%. Two patients of PD status achieved complete remission (CR), one patient of PD status achieved partial remission, two patients with residual lesions after chemotherapy achieved CR, and four patients had no response. The objective remission rate was 55.6%. After the median follow-up of 14.5 months, five patients died, and three patients were completely relieved while one patient was lost during follow-up. The 3-year overall survival was 44.4% and 3-year progression-free survival was 33.3%. EBV-specific antigen peptide-activated CTLs showed positive effect in certain patients with refractory and recurrent AITL with high clinical safety.

RATIONALE:Myeloid sarcomas (MS) are defined as rare extramedullary masses composed of immature myeloid cells. MS mostly develops in patients with acute myeloid leukemia (AML), and involves primarily the skin, soft tissues, bones, and lymph nodes. Pleura and pericardium involvement of MS are extremely uncommon. Polyserositis is also a very rare extramedullary presentation of acute myeloid leukemia (AML).PATIENT CONCERNS:A 30-year-old woman with a complaint of right neck mass combined with coughing for 2 months as well as fever and systemic edema for the last 10 days, was admitted to our center on July 11, 2019. Initial positron emission tomography (PET) scan indicated systemic lymphadenopathy, bilateral pleural effusion, and pericardial effusion.DIAGNOSIS:The initial pathological diagnosis of lymph nodes was MS. Subsequent bone marrow analysis confirmed AML.INTERVENTIONS:Conventional IA induction regimen followed by high-dose cytarabine (HiDAC) regimen.OUTCOMES:Complete absorption of pericardial and pleural effusion after the first cycle of IA induction chemotherapy.LESSONS:Polyserositis can be an extramedullary presentation of AML. Patients with polyserositis should undergo routine flow cytometric analysis. For AML with extamedullary infiltration, systemic chemotherapy should be administered in all confirmed cases.

Background: Multiple myeloma has different kinds of renal injuries. C3 glomerulonephritis (C3GN) is characterized by abnormal deposition of complement C3 in the glomeruli due to abnormal activation of the alternative pathway of complement system. The association between C3GN and multiple myeloma had been well established. However, the mild renal injury of C3GN in the multiple myeloma patients with high level of light chain had not been reported.Case presentation: A 55 years old Chinese man presented with proteinuria. Combined with immunofixation electrophoresis, bone marrow biopsy and renal biopsy, this patient was diagnosed with IgA type multiple myeloma accompanied by C3GN and light chain tubular reabsorption. Although he had a higher level of lamda serum free light chain, the renal injury of this patient was mild. After treated with 4 courses of BD, one course of PAD and autologous stem cell transplantation, he achieved a very good partial hematologic response with stable renal function. Conclusions: In multiple myeloma, the condition for light chain to result in renal impairment of C3GN is that the light chain reaches a certain level and lasts for a long time. Early diagnosis and early intensive treatment are extremely important for the prognosis of such patients.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy arising from plasmacytoid dendritic cell precursors. The disease typically manifests in the skin, but it also evolves into a leukemic phase or can be complicated by other myeloid malignancies, especially myelomonocytic tumors. The association between these neoplasms is not fully elucidated. We report a case of BPDCN with a history of cytopenia that was supposed to be chronic myelomonocytic leukemia. The patient received intensive chemotherapy and achieved complete remission, but soon relapsed. The successive occurrence of myelomonocytic neoplasm and BPDCN is in accordance with the fact that they evolve from a common cell origin with a multilineage potential for myelomonocytic and plasmacytoid dendritic cell differentiation. This case may shed further light on the mystery of biology and the histogenesis of BPDCN.

Immortalized cell lines are useful for deciphering the pathogenesis of acute leukemia and developing novel therapeutic agents against this malignancy. In this study, a new human myeloid leukemia cell line YBT-5 was established. After more than 1-year cultivation from the bone marrow of a patient with acute monocytic leukemia, YBT cell line was established. Then a subclone, YBT-5, was isolated from YBT using single cell sorting. Morphological and cytogenetical characterizations of the YBT-5 cell line were determined by cytochemical staining, flow cytometry analysis, and karyotype analysis. Molecular features were identified by transcriptomic analysis and reverse transcription-polymerase chain reaction. To establish a tumor model, 5 x 10(6) YBT-5 cells were injected subcutaneously in nonobese diabetic/severe combined immune-deficiency (NOD/SCID) mice. DOT1L has been proposed as a potential therapeutic target for KMT2A-related leukemia; therefore, to explore the potential application of this new cell line, its sensitivity to a specific DOT1L inhibitor, EPZ004777 was measured ex vivo. The growth of YBT-5 does not depend on granulocyte-macrophage colony-stimulating factor. Cytochemical staining showed that alpha-naphthyl acetate esterase staining was positive and partially inhibited by sodium fluoride, while peroxidase staining was negative. Flow cytometry analysis of YBT-5 cells showed positive myeloid and monocytic markers. Karyotype analysis of YBT-5 showed 48,XY,+8,+8. The breakpoints between KMT2A exon 10 and exon 11 (KMT2A exon 10/11) and MLLT3 exon 5 and exon 6 (MLLT3 exon 5/6) were identified, which was different from all known breakpoint locations, and a novel fusion transcript KMT2A exon 10/MLLT3 exon 6 was formed. A tumor model was established successfully in NOD/SCID mice. EPZ004777 could inhibit the proliferation and induce the differentiation of YBT-5 cells. Therefore, a new acute monocytic leukemia cell line with clear biological and molecular features was established and may be used in the research and development of new agents targeting KMT2A-associated leukemia.

OBJECTIVE:To investigate the expression of CD160 on the surface of human natural killer (NK) cells and its possible relationship with hematological malignancies.METHODS:CD160 expression on human leukemia cell line NK92 cells was confirmed by reverse transcription-polymerase chain reaction (RT-PCR) and Western blot. The proliferation characteristics and cell surface markers of this cell line were determined. Cytotoxicity of NK92 against 2 human myeloid leukemia cell lines, K562 and THP-1 was analyzed ex vivo. CD160 blocking antibody CL1-R2 was employed to clarify its role in NK cell mediated cytolysis. Then, the expression of CD160 on NK cells in peripheral blood from various patients with hematological malignancies were measured by flow cytometry.RESULTS:The mRNA and protein levels of CD160 expressions on NK92 cells were confirmed by RT-PCR and Western blot, respectively. The flow cytometry results demonstrated that the strong positive expression of CD160 could be detected on the NK92 cell surface. NK92 could effectively kill K562 and THP-1 cells, while the cytolysis effect was abrogated in the presence of CD160 blocking antibody CL1-R2. The high levels of HVEM were expressed on both target cells, but the HLA class I molecules were absent on K562. The expression of CD160 on CD3-CD56+ NK cells in peripheral blood from patients with acute myeloid leukemia (AML), chronic lymphocytic leukemia (CLL) and allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients was significant lower than that in the normal controls (P<0.05).CONCLUSION:The cytolysis function of human NK cells is mediated partially by CD160 molecule. The decrease of CD160 expression on NK cells from patients with various hematological malignancies implies that down-regulation of CD160 expression may be a novel mechanism of tumor immune escape.

Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. To evaluate the efficacy of rituximab in this rare and heterogeneous disease, clinical and pathological data from 27 cases of iMCD were retrospectively analyzed from two large medical centers in China. The novel diagnostic criteria for iMCD were applied, and POEMS syndrome, IgG4-related diseases, and follicular dendritic cell sarcomas cases were excluded from analyses. Total response rate of rituximab- and cyclophosphamide-containing regimens was 55.5%, with 33.3% (9/27) of the cases reaching CR and 22.2% (6/27) PR. In the 14 cases of R-R iMCD, total response rate was only 42.9% (CR 14.3% [2/14], PR 28.6% [4/14]). The 5-year OS of these 27 iMCD cases was 81% (95% CI 64–98; 27 total patients, 4 events, 23 censored) after receiving these regimens, but the 5-year PFS was 43% (95% CI 19–66; 25 total patients, 11 events, 14 censored). Thus, rituximab-based regimens should be considered for the treatment of iMCD patients when siltuximab is not available and potentially in siltuximab-refractory cases.