BackgroundPrimary thyroid lymphoma (PTL) is an extremely rare malignancy, and its prognosis primarily depends on factors such as histological type and clinical stage. Papillary thyroid carcinoma(PTC) is the most common pathological type of thyroid cancer. Papillary thyroid microcarcinoma(PTMC) accounts for a significant proportion of PTCs and generally has a favorable prognosis;however, central lymph node metastasis (CLNM) occurs in approximately 10-30% of cases. Thecoexistence of PTL and metastatic PTMC is exceedingly rare, with only a few reports in the literature. Hashimoto thyroiditis (HT) is a common risk factor for both PTL and PTC.MethodsWe present a case of HT diagnosed concurrently with PTL and PTMC, accompanied by level VI CLNM. We systematically reviewed the patient’s clinical presentation, imaging findings, laboratory results, histopathological features, treatment, and follow-up.ResultsA 40-year-old woman presented with neck discomfort. Ultrasonography revealed a solid hypoechoic nodule in the right thyroid lobe with irregular and slightly lobulated margins. The patientdeclined fine-needle aspiration cytology and opted for direct surgery. The patient underwent right thyroid lobectomy with isthmusectomy and biopsy of the right central compartment (level VI) lymph nodes. Postoperative pathology confirmed the coexistence of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), with pathological features suggestive of large B-cell transformation, and PTMC, with carcinoma identified in one of ten resected level VI lymph nodes (1/10). Immunohistochemistry showed that the lymphoma cells were positive for CD20, CD79a, Pax-5, and Bcl-2 and negative for CD5, CD10, and Cyclin D1. The patient received four cycles (six infusions) of a single-agent, obinutuzumab, as post-surgical therapy. The patient has now completed the full treatment course, remains in a stable condition, and shows no evidence of recurrence.ConclusionThe coexistence of PTL and PTMC is exceptionally rare and poses a significant risk of misdiagnosis. Comprehensive preoperative evaluation and precise histopathological examination are crucial for accurate diagnosis. Although PTMC generally has an excellent prognosis, central lymph node metastasis can occur in a subset of patients. Careful evaluation of suspicious cervical lymph nodes and individualized postoperative surveillance are therefore warranted. Therapeutic decision-making requires a balanced consideration of lymphoma stage and thyroid carcinoma risk stratification, underscoring the importance of a multidisciplinary approach.

BACKGROUND:The tumour-stroma ratio (TSR) is a potential prognostic indicator, yet hindered by quantification challenges and conflicting reports. OBJECTIVE:To determine whether TSR follows a non-linear prognostic pattern and to develop an artificial intelligence (AI)-powered framework for standardised TSR assessment and prognosis prediction in hepatocellular carcinoma (HCC). DESIGN:We integrated whole-slide image (WSI) data with clinical variables across a retrospective cohort (n=392) and The Cancer Genome Atlas dataset (n=168). Restricted cubic splines were used to interrogate non-linear hazard dynamics, with biological validation via transcriptomics and immunohistochemistry. An AI-driven foundation model framework was developed for TSR quantification and multimodal prognostic modelling. RESULTS:Our analysis unveiled an inverted U-shaped non-linear relationship between TSR and mortality, identifying a risk initiation threshold at 0.188 and a peak at 0.268. Transcriptomics analysis indicated that this high-risk phenotype is characterised by active tumour proliferation, stromal activation and tumour microenvironment crosstalk. Technically, AI-derived TSR showed strong correlation with expert assessment (R² >0.9). Furthermore, we developed a novel 'Token-Guided Multimodal Fusion' architecture to integrate WSI, TSR and clinical variables as high-dimensional tokens directly into the computational logic. Consequently, our multimodal framework demonstrated prognostic accuracy (area under the curve >0.80) compared with unimodal baselines. CONCLUSION:This study redefines TSR assessment, shifting from manual estimation to high-dimensional semantic reasoning. By identifying the non-linear prognostic mechanics of the stroma, our token-guided framework offers a biologically interpretable solution for HCC. We suggest that the future of computational pathology may lie not in simple quantification, but in the semantic fusion of human domain knowledge with AI reasoning.

Background: Pancreatic stellate cell (PSC) activation drives fibrogenesis in alcoholic chronic pancreatitis (ACP). While cytokines and growth factors are known regulators of PSC activation. This study investigated the role of TGF-β1/Smad pathway in ethanol (EtOH)-induced human PSC activation and collagen type I alpha 1 chain ( Col1A1) synthesis, as well as the mechanism through which all‑trans retinoic acid (ATRA) antagonizes this pathway. Methods Using human activated PSC line (HP-1 cells), we quantified the mRNA and protein levels of TGF-β1, IL-6, α-SMA and Col1A1 by qRT-PCR and ELISA respectively. Phospho-Smad2/3 and RARα/RARγ levels were analyzed by Western blot, RARα/α-SMA localization by double immunofluorescence, and serum retinol by HPLC. Results EtOH significantly upregulated TGF-β1 and IL-6 mRNA and protein levels. EtOH-induced TGF-β1 upregulation was partially inhibited by si-IL-6R or IL-6 antibody, indicating that IL-6 acts as an intermediate. Crucially, both TGF-β1 siRNA and a neutralizing antibody largely abolished EtOH-induced Smad2/3 phosphorylation and the subsequent production of α-SMA and Col1A1, underscoring the central role of TGF-β1 in this pathway. ATRA suppressed α-SMA expression and prevented Col1A1 synthesis in EtOH-treated HP-1 cells without affecting RARα. Mechanistically, these effects occurred via partial inhibition of TGF-β1 and marked downregulation of Smad2/3 phosphorylation. Clinically, a high vitamin A deficiency rate (44.7%) and elevated serum TGF-β1/Col1A1 levels were observed in 38 ACP patients. Conclusion ATRA mitigates EtOH-induced fibrogenesis in PSCs through inhibition of the TGF-β1/Smad2/3 axis. This mechanistic insight, together with the observed vitamin A deficiency in ACP patients, suggests that restoring retinoid signaling may be a viable antifibrotic strategy.

Accurate assessment of human epidermal growth factor receptor 2 (HER2) expression is foundational for targeted therapy in breast cancer (BC). The recent expansion of treatment eligibility to include HER2-ultralow poses a significant diagnostic challenge due to poor inter-observer reproducibility. We developed and validated a whole-slide image (WSI)-based deep learning (DL) model to standardize the identification and quantification of HER2-ultralow expression. A DL model was developed using a single-center training set. For external validation, an initial pool of 180 cases (originally archived as HER2 IHC 0 or 1+) was screened across 20 medical centers. Following rigorous quality control and expert consensus re-evaluation, a high-quality validation cohort of 89 cases (66 primary, 23 metastatic) was selected. The final consensus labels for this cohort included 65 cases of IHC 0, 19 of IHC 1+, and 5 of IHC 2+ (all ISH-negative). To address inter-pathologist variability, a reference standard was established through consensus review by expert breast pathologists, supplemented by Gaussian kernel density estimation (KDE) for continuous quantification of ultralow signals. Performance was benchmarked against participating pathologists using recall, F1 score, and mean absolute error (MAE). The AI model demonstrated superior diagnostic performance in IHC 0 classification compared to pathologists, with higher overall recall (0.862 vs. 0.828) and F1 score (0.761 vs. 0.755). Critically, the model achieved a 93.8

Paraquat (PQ) is a widely used herbicide known to induce lung injury and fibrosis in humans. Naringin (Nar), a naturally occurring flavonoid, possesses anti-inflammatory and anti-fibrotic properties. This study explores the protective mechanisms of Nar against PQ-induced epithelial-mesenchymal transition (EMT) and pulmonary fibrosis, focusing on the N-Myc downstream-regulated gene 1 (NDRG1) and peroxisome proliferator-activated receptor gamma (PPARγ) signaling pathways. Results demonstrate that Nar treatment significantly suppresses the PQ-mediated increase in EMT and pro-fibrotic markers, such as TGF-β, Vimentin, Snail, N-cadherin, and α-SMA, while also reducing the release of pro-inflammatory and pro-fibrotic factors, including IL-6, TNF-α, IL-1β, TGF-β, and MMP-9. Additionally, Nar decreases NDRG1 levels and enhances PPARγ expression. Mechanistically, NDRG1 silencing led to diminished JNK activity, which restored PPARγ levels and alleviated the expression of EMT markers and cytochrome P450s (CYPs 1A2 and 2E1) in A549 cells. Furthermore, Nar effectively mitigated PQ-induced EMT, cellular migration, and invasion by downregulating NDRG1 and phosphorylated JNK while upregulating PPARγ. These findings highlight the critical involvement of the NDRG1/JNK/PPARγ signaling pathway in PQ-induced lung injury and suggest the therapeutic potential of Nar for pulmonary fibrosis.

Solid tissue biopsy is fundamental in guiding surgeons during intraoperative and peri-operative management of cancer patients. However, conventional histopathologic methods depend heavily on the expertise of trained pathologists, facing challenges in accuracy and efficiency. Methods: Here, we show that unbiased labeling of proteins within tissue sections using tumor-selective dyes enhances tumor-specific signals, enabling robust and accurate differentiation of tumors from normal tissues in less than 45 min. This diagnostic approach combines a tumor-selective dye labeling strategy and a three-dimensional (3D) histological electrophoresis separation strategy to visualize protein differences between tissues and exclude off-target interference. Results: We successfully diagnose and delineate malignant tissue from frozen and fresh surgical specimens from 34 patients across six types of cancer (mean AUC = 0.93). Furthermore, we apply this method to distinguish different histological characteristics in liver cancer surgical specimens, as well as identify and quantify the degree of inflammation in tumor-surrounding tissues. Conclusion: This rapid, accurate, unbiased, and marker-free approach may enhance intraoperative detection of multiple types of tumor specimens.

Histopathological diagnosis is a crucial part of surgical treatment, as it determines further treatment decisions and prognosis for patients. Due to the heterogeneity of tumors, traditional histopathological diagnosis relies on histological and cytological morphological analysis of cryo-slides by well -trained pathologists, which is subjective, time-consuming, and lacks considerable reliability. Therefore, we engineer a series of tumor receptor -seeking dyes to automatically label and visually depict the tumor contour with antibody -free interaction. The meso-chlorine on the cyclohexyl ring of dyes can covalently react with specific thiol groups in various tumor signature proteins to form stable dye@protein complexes. These dye@protein complexes have significantly amplified fluorescence signals in tumor sites, enabling highresolution delineation of tumor margins and providing histological morphology information to potentially determine tumor staging. The orthogonal effect not only avoids errors caused by individual signature proteins, but also amplifies the fluorescence difference between tumor and normal tissues. Notably, our method is easy to operate with a minimal detection time of only 4 min. The tumor receptor -seeking dyes successfully assess tumor -positive margins from 66 resected breast tumor samples and 25 intraoperative cancer patients with 100% accuracy, providing a newgeneration technique for intraoperative pathologic diagnosis of tumor biopsy.

Background Sepsis-induced acute lung injury (ALI) leads to severe hypoxemia and respiratory failure, contributing to poor prognosis in septic patients. Endotoxin dissemination triggers oxidative stress and the release of inflammatory cytokines in macrophages, initiating diffuse alveolar damage. The role of epigenetic histone modifications in organ injury is increasingly recognized. The present study aimed to investigate the use of a histone modification inhibitor to alleviate sepsis-induced ALI, revealing a new strategy for improving sepsis patient survival.Methods In vivo models of ALI were established through the intraperitoneal injection of lipopolysaccharide and cecal ligation and puncture surgery. Furthermore, the disease process was simulated in vitro by stimulating Tamm-Horsfall protein-1 (THP-1) cells with lipopolysaccharide. Hematoxylin and eosin staining, blood gas analysis and pulmonary function tests were utilized to assess the extent of lung tissue damage. Western blot analysis, real-time polymerase chain reaction, enzyme-linked immunosorbent assay and immunofluorescence were used to measure the levels and distribution of the indicated indicators within cells and tissues. Reactive oxygen species and autophagic flux alterations were detected using specific probes.Results BRD3308, which is a inhibitor of histone deacetylase 3, improved lung tissue damage, inflammatory infiltration and edema in ALI by inhibiting Nod-like receptor protein3-mediated pyroptosis in macrophages. By upregulating autophagy, BRD3308 improved the disruption of redox balance in macrophages and reduced the accumulation of reactive oxygen species. Mechanistically, BRD3308 inhibited histone deacetylase 3 activity by binding to it and altering its conformation. Following histone deacetylase 3 inhibition, acetylation of H3K27 was significantly increased. Moreover, the increase in H3K27Ac led to the upregulation of autophagy-related gene 5, a key component of autophagosomes, thereby activating autophagy.Conclusions BRD3308 inhibits oxidative stress and pyroptosis in macrophages by modulating histone acetylation, thereby preventing sepsis-induced ALI. The present study provides a potential strategy and theoretical basis for the clinical treatment of sepsis-induced ALI. Graphical Abstract

Tissue diagnosis is important during surgical excision of solid tumors for margin evaluation. Conventional histopathologic methods rely heavily on image-based visual diagnosis by specialized pathologists, which can be time-consuming and subjective. We report a three-dimensional (3D) histological electrophoresis system for rapid labeling and separation of the proteins within tissue sections, providing a more precise assessment of tumor-positive margin in surgically resected tissues. The 3D histological electrophoresis system uses a tumor-seeking dye labeling strategy to visualize the distribution of tumor-specific proteins within sections and a tumor finder that automatically predicts the tumor contour. We successfully demonstrated the system's capability to predict the tumor contours from five murine xenograft models and distinguish the tumor-invaded region of sentinel lymph nodes. Specifically, we used the system to accurately assess tumor-positive margins from 14 patients with cancer. Our 3D histological electrophoresis system serves as an intraoperative tissue assessment technology for more accurate and automatic pathologic diagnosis.

OBJECTIVE:Selecting interventions for patients with solitary hepatocellular carcinoma (HCC) remains a challenge. Despite gross classification being proposed as a potential prognostic predictor, its widespread use has been restricted due to inadequate studies with sufficient patient numbers and the lack of established mechanisms. We sought to investigate the prognostic impacts on patients with HCC of different gross subtypes and assess their corresponding molecular landscapes.DESIGN:A prospective cohort of 400 patients who underwent hepatic resection for solitary HCC was reviewed and analysed and gross classification was assessed. Multiomics analyses were performed on tumours and non-tumour tissues from 49 patients to investigate the mechanisms underlying gross classification. Inverse probability of treatment weight (IPTW) was used to control for confounding factors.RESULTS:Overall 3-year survival rates varied significantly among the four gross subtypes (type I: 91%, type II: 80%, type III: 74.6%, type IV: 38.8%). Type IV was found to be independently associated with poor prognosis in both the entire cohort and the IPTW cohort. The four gross subtypes exhibited three distinct transcriptional modules. Particularly, type IV tumours exhibited increased angiogenesis and immune score as well as decreased metabolic pathways, together with highest frequency of TP53 mutations. Patients with type IV HCC may benefit from adjuvant intra-arterial therapy other than the other three subtypes. Accordingly, a modified trichotomous margin morphological gross classification was established.CONCLUSION:Different gross types of HCC showed significantly different prognosis and molecular characteristics. Gross classification may aid in development of precise individualised diagnosis and treatment strategies for HCC.

Purpose Atypical teratoid/rhabdoid tumour (AT/RT) is a highly malignant central nervous system tumour of early childhood. According to the latest WHO classification, the diagnosis of AT/RTs needs to be confirmed by the absence of SMARCB1 (INI1) or SMARCA4 (BRG1) protein expression. AT/RT in the pineal region is infrequent and most have not been proven genetically. Here, we report a case of AT/RT in the pineal region, preoperatively misdiagnosed as a meningioma. Immunohistochemistry revealed the absence of INI1 protein expression. Method A 29-month-old boy was admitted to the hospital after 14 days of emotional apathy and a 2-day vomiting history. AT/RT was not considered during the initial diagnosis because this tumour is rare in this region and is often accompanied by cystic degeneration and necrosis on imaging. Subsequently, the patient underwent surgery and the tumour was completely excised. Result The pathological diagnosis was AT/RT. After discharge, the patient continued chemotherapy in other hospitals but died five months after surgery because of disease progression. Conclusion To our knowledge, this is the fifth case of paediatric pineal AT/RT confirmed genetically. Although in children AT/RT in the pineal gland is rare, a differential diagnosis of AT/RT should be considered when new pineal masses appear in children. For this highly malignant disease with poor prognosis, it is very important to detect and recognize the disease as soon as possible, and to adopt surgery plus multiple treatment management.

目的 通过对单中心209例次移植肝穿刺活检组织的病理资料回顾性分析,研究肝移植术后常见并发症的发生情况、病理学改变及鉴别诊断.方法 2013年8月至2023年4月在吉林大学第一医院器官移植中心145例患者共行移植肝穿刺活检209次,采用快速石蜡包埋和制片技术流程,常规行HE染色、Masson、D-PAS及网状纤维等组织化学染色和CK7、CMV、C4d等免疫组织化学染色,EBER原位杂交检测EBV感染.结果 急性T细胞介导的排斥反应最常见,占36.84%,其次为药物性肝损伤,占23.44%,第3位为胆管并发症,占14.35%,此外还有乙型和丙型肝炎病毒感染或复发、缺血/再灌注损伤、巨细胞病毒感染、慢性排斥反应、富于浆细胞的排斥反应、血管并发症、原发病复发、移植肝原发无功能及难以诊断的肝形态.病理学改变:急性T细胞介导的排斥反应的诊断基于汇管区炎症、胆管上皮的炎性损害及静脉内皮炎,其中58.44%的病例可见经典的汇管区"三联征",药物性肝损伤最多见的病理改变为Ⅲ带为主的肝细胞变性、脂肪变性、肝细胞及毛细胆管内的胆汁淤积,胆管并发症表现为肝细胞和毛细胆管内胆汁淤积,汇管区内沿着界面分布的小胆管增生,增生胆管周围可见以中性粒细胞为主的炎细胞浸润,间质水肿.结论 病理医师需要结合患者的临床表现、实验室检查、影像学资料、用药史等资料综合分析并与临床医师充分沟通讨论后作出病理诊断.

Accurate identification of tumor margins during cancer surgeries relies on a rapid detection technique that can perform high-throughput detection of multiple suspected tumor lesions at the same time. Unfortunately, the conventional histopathological analysis of frozen tissue sections, which is considered the gold standard, often demonstrates considerable variability, especially in many regions without adequate access to trained pathologists. Therefore, there is a clinical need for a multitumor-suitable complementary tool that can accurately and high-throughput assess tumor margins in every direction within the surgically resected tissue. We herein describe a high-throughput three-dimensional (3D) histological electrophoresis device that uses tumor-specific proteins to identify and contour tumor margins intraoperatively. Testing on seven cell-line xenograft models and human cervical cancer models (representing five types of tissues) demonstrated the high-throughput detection utility of this approach. We anticipate that the 3D histological electrophoresis device will improve the accuracy and efficiency of diagnosing a wide range of cancers.

移植后淋巴组织增殖性疾病(PTLD)是移植术后比较严重的并发症之一。本文回顾性分析了吉林大学第一医院2013年12月至2022年12月期间完成的肝移植手术后发生淋巴组织增殖性疾病的3例患者临床资料，发病时间均在移植术后1年内。临床表现为发热、淋巴结肿大、消化系统症状及肝功能异常。2例为单形性PTLD，1例为非损毁性PTLD。其中2例存在EB病毒感染。本文分析患者的临床资料及病理学特点，以期为PTLD的早预防、早诊断及规范治疗提供经验。

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm of neural crest origin that predominantly involves the craniofacial region, involvement of the epididymis being extremely rare, with about 30 cases reported. We report an unusual case of a 5-month-old male with MNTI in the epididymis. The patient underwent orchiectomy. Half a year later, there was no sign of recurrence. Whether preoperative examination or intraoperative frozen examination, the tumor may easily be misdiagnosed as malignancy. Melanotic neuroectodermal tumor of infancy should be included in differential diagnosis in infants presenting with fast-growing scrotal swelling.

胆囊动脉假性动脉瘤(GAP)在临床上极为罕见。本文报道1例GAP患者，因突然昏倒急诊入院。影像学检查提示GAP破裂出血，存在血管变异。经胆囊动脉栓塞术，超声引导下胆囊穿刺引流术，腹腔镜下胆囊切除术治疗后，患者康复出院。术后病理证实GAP形成，慢性胆囊炎伴出血性梗死及急性化脓性炎症。

A 45-year-old man presented with a free-floating cyst in the anterior chamber of his left eye (Fig A-B). Medical history revealed a blunt trauma by firework to the left eye 10 years prior. Anterior-segment OCT scan revealed a cyst in the anterior chamber with hyperreflective walls and clear hyporeflective lumen (Fig C-D). The optical density is comparable with the anterior chamber fluid. The cyst was examined by a pathologist, which resulted in discovery of an iris cyst derived from iris pigment epithelium (hematoxylin and eosin staining) (Fig E).

Purpose: Gastric cancer (GC) remains a prevalent aggressive tumor with high morbidity and mortality globally. The identification of GC subtypes based on molecular features improved the prediction of prognosis and the selection of targeted therapies. PTEN is a characteristic tumor suppressor, while its association with different GC subtypes was unknown.Patients and Methods: The cohort consisted of 248 patients diagnosed with gastric cancer who were hospitalized and received radical gastrectomy. In addition, PTEN gene expression matrix of STAD was retrieved from TCGA. The mRNA and protein levels of PTEN and PD-L1 were detected using qRT-PCR and IHC staining. Multivariate logistic regression and Kaplan-Meier analysis were used to examine the relationship between PTEN expression and clinical characteristics.Results: In our study, PTEN was downregulated in gastric tumors both in mRNA and protein levels. Its inactivation was closely linked to higher histological grade (P = 0.005), neural invasion (P = 0.012), depth of invasion (P = 0.021), lymph metastasis (P = 0.026), and TNM stage (P = 0.001) of GC in the present study. Moreover, according to the molecular subtypes, high PTEN expression was related to high TPS score of PD-L1 positively (P = 0.010) but was not associated with MSI and EBV infection. Further, TCGA data validated that PTEN was indeed correlated with histological grade and invasion depth and positively related to PD-L1 expression (R = 0.29, adjusted P < 0.001).Conclusion: The above results suggested that PTEN expression was a useful marker in gastric carcinogenesis and progression and in the selection of immunotherapy-based treatments for GC patients.

Abstract Background: Brentuximab vedotin (BV) showed high overall remission rate in refractory/relapsed classical Hodgkin's lymphoma (HL) and systemic anaplastic large cell lymphoma (sALCL). Although the efficacy of BV has been reported in clinical trials, its effectiveness in real-world treatment settings for patients with CD30 positive, aggressive sub types of non-Hodgkin's lymphoma (NHL) like peripheral T-cell lymphoma with T-follicular helper cell (TFH) phenotype (PTCL, TFH), anaplastic large-cell lymphoma (ALCL) and angioimmunoblastic T-cell lymphoma (AITL) in China has not been documented. Methods Analysis of a real-world, observational, retrospective case series in patients suffering from AITL, sALCL and PTCL-TFH treated with BV in different treatment lines was conducted. The patients were given treatment from May 2020 till 28 th June, 2021. All patients were pathologically diagnosed as PTCL before treatment and expressed CD30. Patients received BV (1.8 mg/kg) combined with CHP (cyclophosphamide, epirubicin, prednisone acetate every 3 weeks). The primary endpoint was the best curative effect and secondary endpoints were objective response rates (ORR), duration of remission, and incidence of adverse events (AEs). The expression level of CD-30 and its correlation with therapeutic effect was also evaluated. Results Out of a total of 21 patients (8 ALCL, 9 AITL, and 1 PTCL-TFH, 2 NK/T, 1 Mycosis fungoides (MF)), 18 patients (16 treatment naive, 2 relapsed) who completed ≥4 cycles of BV-CHP treatment were included for the evaluation of clinical effectiveness. Sixteen patients (90.6%) were identified with Ann Arbor stage III/IV, 14 (77.8%) patients had B symptoms (fever, drenching night sweats and loss of more than 10 percent of body weight over 6 months) and 14 (77.8%) patients had extra nodal infiltration. Further 12 patients had an International Prognostic Index (IPI) score of ≥3 and 13 patients (72.2%) had higher lactate dehydrogenase (LDH) values (table 1). All the patients had higher than normal β2-MG value (100%), which suggests that the patients may not derive benefits from monotherapy with chemotherapeutic agents. The overall ORR was 88.9% (CR:61.1%; PR: 27.8%). In the treatment naïve group, the ORR was 87.5% (CR: 62.5%; PR:25%), among whom the patients with ALCL had the best curative effect (CR 100%), with a duration of response of up to 8 months. In treatment naïve patients with AITL, the best response rate was 87.5%, while one patient had disease progression after 6 cycles of BV+CHP. The overall ORR in treatment naïve patients with AITL was 75% (CR: 25%; PR:50%), with the duration of response of up to 6 months. In the R/R group, ORR reached 100% (CR:50%; PR:50%) (Table 1). The CD30 expression level of ALCL was relatively high, and 80% of them had expression levels above 80%. The use of BV has achieved very significant curative effects. However, in AITL, the overall expression level was ranging from 2%-40%, and there is no obvious correlation with the therapeutic effect. The most common adverse effects occurring in 5% of the patients were peripheral neuropathy, but no patients had neuropathy of grade 3 or above; the second most common adverse reaction was gastrointestinal reactions, including vomiting and diarrhea, especially in the AITL group. Conclusion BV is a promising treatment in patients with ALCL, AITL and PTCL-TFH in both first and refractory treatment settings. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Primary biliary cirrhosis (PBC)–autoimmune hepatitis (AIH) overlap syndrome is frequently associated with extrahepatic autoimmune disorders. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disease that is characterized by complement-mediated hemolysis due to erythrocyte membrane defects. However, autoimmune liver disease was not previously reported to be associated with PNH. A 37-year-old female patient was referred to our hospital with elevated liver enzymes and hematuria. On the basis of the symptoms and results of laboratory tests, radiographic studies, and pathologic results, she was diagnosed with PBC–AIH overlap syndrome and PNH. She was treated with a combination of ursodeoxycholic acid and prednisolone. The patient was symptom-free, with laboratory findings within near-normal range. The patient had recovered well at the 24-month follow-up evaluation. While we acknowledge that this was a single case, these findings expand our knowledge of immunological diseases that are associated with PNH and suggest an immune-mediated pathogenic pathway between PNH and PBC–AIH overlap syndrome. The combination of ursodeoxycholic acid and prednisolone can achieve therapeutic success. Routine follow-up of these patients is necessary to document disease progression.