Mucosa-associated Lymphoid Tissue Lymphoma Research Articles

The National Landscapes of Gastric Mucosa-Associated Lymphoid Tissue Lymphoma: Stable Trends in Black Populations and Late-Stage Tumors.

Helicobacter pylori (H. Pylori) eradication has been the mainstream for preventing and treating gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Prior data showed disparities in eradication rates of H. Pylori between different populations. This can potentially impact the occurrence of gastric MALT lymphoma. There are limited data on the incidence and mortality rates and trends of gastric MALT lymphoma in the US. Therefore, the aim of the current study was to conduct a time-trend analysis of gastric MALT lymphoma incidence and mortality rates in different populations. The incidence rates of gastric MALT lymphoma were calculated from the United States Cancer Statistics (USCS) database (which covers nearly 98% of the US population) between 2001-2020 and were age-adjusted to the standard 2000 US population using SEER*Stat software (version 8.4.3, national cancer institute "NCI"). Incidence-based mortality (IBM) rates, also age-adjusted to the standard 2000 US population, were calculated from the Surveillance Epidemiology and End Results (SEER) database. Tumor location was specified using ICD-O-3 codes C 160-C 169 with malignant behavior. Histopathology was specified using the ICD-O-3 code 9699. The rates were categorized by sex, age, race/ethnicity, and tumor stage at diagnosis. Age groups were older adults (aged 55 years or older) and younger adults (aged younger than 55 years). Race/ethnic groups included Non-Hispanic White (White), Non-Hispanic Black (Black), Hispanic, Non-Hispanic Asian/Pacific Islander (API), and Non-Hispanic American Indian/Alaska Native (AI/AN), as reported in the database. Stage at diagnosis included early stage (in situ and localized tumors) and late stage (regional and distant site tumors). Joinpoint Regression Software (version 5.0.2, NCI) using the weighted Bayesian Information Criteria method was used to generate time trends. Trends were reported as annual percentage change (APC) and average APC (AAPC). Parametric estimations were used with a two-sided t-test to evaluate the trends with a p-value cutoff at 0.05. There were 21,625 patients diagnosed with gastric MALT lymphoma in the US between 2001 and 2020. Overall, incidence rates were significantly decreasing over the study period (AAPC = -1.93). This decrease was seen in males (AAPC = -1.67) and in females (AAPC = -1.66) (Figure). When categorized by age groups, older adults also experienced a significant decrease in gastric MALT lymphoma incidence rates (AAPC = -1.66). While this was also seen in younger adults, the rates were decreasing at a slower pace (AAPC = -1.38). When categorizing the trends by race/ethnicity, incidence rates were significantly decreasing in White (AAPC = -2.09), Hispanic (AAPC = -1.61), and API (AAPC = -3.92) populations. However, the rates were stable among Blacks. While early-stage tumors experienced a significant decrease (AAPC = -1.10), the rates were stable for late-stage tumors. When evaluating mortality, there were 11,036 patients whose death was attributed to gastric MALT lymphoma between 2000 and 2020. IBM rates were decreasing in males (AAPC = -1.47), older adults (AAPC = -1.55), Whites (AAPC = -1.23), Hispanics (AAPC = -1.73), APIs (AAPC = -2.30), and early-stage tumors (AAPC = -1.08). On the other hand, IBM rates were stable in females, younger adults, Blacks, and late-stage tumors. An extensive nationwide data analysis encompassing nearly 98% of patients diagnosed with gastric MALT lymphoma in the US unveils a declining trend in the incidence of cancer overall over the past two decades. This decline is observed in both sexes and various age groups. When stratifying by race and ethnicity, this incidence has been decreasing in all populations except among Black individuals. While early-stage tumors have also demonstrated a significant decrease in incidence rates, late-stage tumors have shown no parallel decline. Mortality evaluation also revealed an improvement in most of the US population except among females, younger adults, Black individuals, and late-stage tumors. While the cause of our findings is unclear, it could be driven by disproportionate exposure to risk factors, including H. Pylori, and disparities in screening, management, and outcomes. Future studies are warranted to investigate factors contributing to worse outcomes of gastric MALT lymphoma, especially in the Black population.

Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland: A case report and literature review.

The most common subtype of primary lymphoma of the ocular adnexa is the mucosa-associated lymphoid tissue (MALT) subtype. MALT lymphoma of the lacrimal gland is relatively rare among the lacrimal gland tumors, and the early clinical symptoms are atypical, which can easily lead to misdiagnosis and missed diagnosis. Here, we report a case of MALT lymphoma of the lacrimal gland and explore its clinical manifestations, pathological characteristics, management, and pathogenesis, with the aim of helping clinicians gain an in-depth understanding of ocular adnexal MALT lymphoma. A 60-year-old man presented to our hospital with proptosis and diplopia. The right eye deviated and shifted toward the lower part of the nose. Orbital enhanced magnetic resonance imaging suggested a mass with a maximum cross-section of 3.2 × 2.1 cm. T1 weighted image was isointense, and the enhancement was more uniform and obvious. The right orbital mass was treated surgically, and the final pathology report was MALT lymphoma. After the pathological report was released, the patient was transferred to the hematology department for further diagnosis and no further treatment was given eventually. Seven months later, the patient did not complain of discomfort. Whole-body positron emission tomography-computed tomography, superficial lymph node examination and orbital magnetic resonance imaging revealed no abnormal changes. The clinical manifestations of MALT lymphoma are heterogeneous. Imaging examination is important for assessing the size of the tumor and its relationship with adjacent tissues. Postoperative pathological examination may provide further evidence for the evaluation of the patient's surgical efficacy and prognosis. Management of MALT lymphoma of the lacrimal gland requires a multidisciplinary approach involving ophthalmologists, hematologists, and radiotherapists.

#1494 Immunotactoid glomerulopathy: a comprehensive analysis of three cases

Abstract Background Immunotactoid glomerulopathy (ITG) is an uncommon cause of glomerular disease encountered in approximately 0.06% of native kidney biopsies. This fibrillary disease is strongly associated with haematologic disorders, including lymphoma (mainly chronic lymphocytic leukaemia/small lymphocytic lymphoma), monoclonal gammopathy, and multiple myeloma. It is usually managed with oral steroids and/or treatment of an underlying lymphoproliferative disorder. Here, we report three cases of ITG, all female patients with varying severity of the disease, timing of malignancy and required treatment. Case Scenarios: Case 1: A 76-year-old lady with no significant medical background presented in May 2020 with confusion and progressive peripheral oedema. She had AKI (serum creatinine 210 µmol/L from baseline 68 µmol/L) and a sodium of 104 mmol/l. Urine PCR was 1182 mg/mmol. She was admitted to the ITU, where hyponatraemia was managed with a good clinical response. Blood screening showed negative immune, myeloma and virology screens. Complement was low. A renal biopsy showed a diagnosis of ITG. She had an extensive malignancy screen, but no malignancy was detected. She was started on prednisolone 60 mg once daily and eventually weaned off the drug by March 2021, as she maintained remission of her disease. Two years after her initial presentation, she was seen by the ophthalmologist for a right lacrimal gland mass. This was excised, and a biopsy revealed a MALT Lymphoma. Case 2: A 50-year-old lady with splenomegaly being investigated under the haematology service was referred to our renal clinic for proteinuria in June 2019. Her serum creatinine was within normal limits, and her UPCR was 302 mg/mmol. She had no paraprotein in her blood, low serum immunoglobulins, low C3 and a negative viral screen. Perindopril, initiated by her GP, was up-titrated. Renal biopsy revealed features in keeping with Immunotactoid Glomerulopathy, and completed haematological investigations revealed Chronic Lymphocytic Leukaemia. Her UPCR peaked at 842 mg/mmol before reducing to 190 mg/mmol within a year after treatment with Veneclotax and Rituximab after not tolerating FCR (Fludarabine, Cyclophosphamide, Rituximab) Chemotherapy. Case 3: A 69-year-old lady presented to the renal clinic with proteinuria in September 2018. She had a background of breast cancer diagnosed in 2007 (She had surgical resection followed by radiotherapy). Her 24-hour urine protein was 2g, and UPCR was 155 mg/mmol. Serum creatinine was 81 µmol/l and eGFR 66 ml/min. P- ANCA was positive, while PR3 and ANA were negative. She had bilateral leg oedema due to long-standing lymphedema. The biopsy report showed immunotactoid glomerulopathy. Regular surveillance of previous breast cancer with mammography and CT-CAP has shown no evidence of malignancy recurrence, hepatitis virology and paraprotein were negative, and C3/C4 were within normal limits. She had improved UPCR and maintained normal kidney function on full-dose Losartan. Conclusion Immunotactoid Glomerulopathy is a rare form of glomerular disease with a strong association with lymphoproliferative malignancies, which may precede, occur concomitantly or present years after the initial diagnosis. This common association highlights the need for diligence in screening for malignancy in each case, and it is a reason for careful follow-up of these patients. Treatment of underlying disorders is associated with positive renal outcomes, and in some cases, there is a good response to steroids.

Molecular Mechanisms and Treatment Strategies for Helicobacter pylori-Induced Gastric Carcinogenesis and Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma.

Helicobacter pylori has been classified as a class I carcinogen by WHO because of its primary involvement in the development of gastric cancer and mucosa-associated lymphoid tissue (MALT) lymphoma. This review focuses on understanding the molecular pathophysiological mechanisms that operate within intracellular transduction pathways and their relevance in the treatment strategies for the two main diseases caused by H. pylori. H. pylori virulence factors such as cytotoxin-associated gene A and vacuolating cytotoxin A genotypes, inflammatory mediators, H. pylori-induced microRNA deregulation, alterations in autophagy proteins and regulators, and changes in DNA methylation are some of the molecular mechanisms that play essential roles in H. pylori infection and gastric carcinogenesis. The discovery of novel treatment strategies that target the deregulated intracellular transduction pathways in gastric carcinogenesis and MALT lymphoma is critical. H. pylori eradication (HPE) is not limited to H. pylori-dependent low-grade MALT lymphoma and may be used in patients with high-grade diffuse large B-cell lymphoma (DLBCL) (de novo or DLBCL-MALT lymphoma). The loss of H. pylori dependency and high-grade transformation appear to be distinct events in the progression of gastric lymphoma. Interestingly, patients with H. pylori-positive gastric DLBCL without histological evidence of MALT lymphoma (pure gastric DLBCL) may respond to HPE therapy.

Clinical characteristics and prognostic analysis of 34 patients with primary thyroid lymphoma

Objective: To investigate the clinical characteristics, diagnosis, treatment, and prognosis of primary thyroid lymphoma (PTL) . Methods: A retrospective analysis was conducted on the clinical and pathological data of 34 newly diagnosed PTL patients admitted to Beijing Tongren Hospital from September 2010 to February 2023. The Kaplan-Meier survival curve and Log-rank test were used for survival analysis, and the Cox regression model was applied for univariate analysis of prognostic factors. Results: All 34 PTL patients presented with cervical mass as the initial clinical manifestation. There were 9 males and 25 females. The pathological diagnosis was diffuse large B-cell lymphoma (DLBCL) in 29 patients and mucosa-associated lymphoid tissue (MALT) lymphoma in 5 patients. Among the DLBCL patients, 6 had B symptoms, 17 had an Eastern Cooperative Oncology Group (ECOG) score of ≥2, the Ann Arbor staging was stage Ⅰ-Ⅱ in 21 cases and stage Ⅲ-Ⅳ in 8 cases, the tumor diameter was ≥10 cm in 4 cases, and 14 had concurrent Hashimoto thyroiditis; 27 cases received chemotherapy, with 21 cases achieving complete remission (CR), 2 cases partial remission (PR), and 6 cases of disease progression; the 5-year progression-free survival and overall survival rates were 78.9% and 77.4%, respectively; univariate survival analysis showed that B symptoms, tumor diameter ≥10 cm, and Ann Arbor stage Ⅲ-Ⅳ were significant factors affecting patient prognosis (P<0.05). MALT lymphoma patients were all in stages Ⅰ-Ⅱ, had an ECOG score of 0-1, and were without B symptoms. All patients underwent surgical resection, with 4 cases achieving CR and 1 case PR. Conclusion: PTL is more common in females with concurrent Hashimoto thyroiditis, with the majority of pathological types being B-cell lymphoma. The main treatment is chemotherapy, supplemented by radiotherapy and surgery, and the prognosis is relatively favorable.

Effect of intestinal microbiota on duck short-beak and dwarf syndrome caused by novel goose parvovirus

Short-beak and dwarf syndrome (SBDS) is caused by infection with novel goose parvovirus (NGPV), which leads to intestinal dysbiosis, developmental delay, short beak, lameness, and paralysis in ducks and is the cause of skeletal health problems. NGPV infection can cause intestinal microbial disturbances, but it is still unclear whether the intestinal microbiota affects the pathogenicity of NGPV. Here, the effects of intestinal microbiota on NGPV-induced SBDS in Cherry Valley ducks were assessed by establishing a duck model for gut microflora depletion/reestablishment through antibiotics (ABX) treatment/fecal microbiota transplanted (FMT). By measuring body weight, beak length, beak width and tarsal length, we found that SBDS clinical symptoms were alleviated in ducks treated with ABX, but not in FMT ducks. Next, we conducted a comprehensive analysis of bone metabolism, gut barrier integrity, and inflammation levels using quantitative real-time PCR (qPCR), enzyme linked immunosorbent assay (ELISA), biochemical analysis and histological analysis. The results showed that ABX treatment improved bone quality reduced bone resorption, mitigated tissue lesions, protected intestinal barrier integrity, and inhibited systemic inflammation in NGPV-infected ducks. Moreover, cecal microflora composition and short-chain fatty acids (SCFAs) production were examined by bacterial 16S rRNA sequencing and gas chromatography. The results revealed that ABX treatment mitigated the decreased abundance of Firmicutes and Bacteroidota in NGPV-infected ducks, as well as increased SCFAs production. Furthermore, ABX treatment reduced the mucosa-associated lymphoid tissue lymphoma translocation protein 1 (Malt1) and nuclear factor κB (NF-κB) expression, which are correlated with systemic inflammation in SBDS ducks. These findings suggested that intestinal microflora depletion alleviated NGPV-induced SBDS by maintaining intestinal homeostasis, inhibiting inflammatory response and alleviating bone resorption. These results provide evidence for the pivotal role of intestinal microbiota in the process of SBDS and contribute a theoretical basis for the feasibility of microecological preparation as a method to control SBDS.

Lower IFN-γ expression causes more severe gastric inflammation in BALB/c infected by Bali 03 Helicobacter pylori isolate that provided by low iron diet

Background: Helicobacter pylori (H. pylori) infection is associated with various gastrointestinal diseases including gastritis, peptic ulcers, gastric carcinoma, and gastric mucosa-associated lymphoid-tissue lymphoma (MALT) lymphoma. Several studies have shown that H. pylori plays a role in iron deficiency that is refractory to iron administration. This study aims to analyze whether the lower IFN-γ expression causes more severe gastric inflammation in BALB/c infected by Bali 03 H. pylori isolate that is provided by a low iron diet. Methods: This research was an experimental study by inoculating the bacterial isolate H. pylori Bali 03 in two groups of BALB/c mice. The treatment group was given a low iron diet at a dose of &lt;3 mg/kgBW of mice, while the control group was given a normal dose of iron diet at a dose of 40 mg/kgBW of mice. The cellular immune response of the two groups of BALB/c mice was measured by comparing the expression of IFN-γ, granzyme B, IFN-γ/IL-10 ratio to the degree of gastric inflammation. Results: The results of the research were that the H. pylori Bali 03 isolate caused inflammation in 36 (100%) gastric of the mice. The degree of gastric inflammation of mice was more severe in the treatment group than in the control group, within 6 weeks precancerous lesions occurred, namely gastric gland atrophy and metaplasia in the treatment group, 1 (2.7%). There is a significant difference between the control group and the treatment group (p=0.019). There was a significant difference in IFN-γ expression between the treatment group and the control group (p=0.034). Conclusion: Iron deficiency affects the degree of gastric inflammation through IFN-γ expression in BALB/c infected with H. pylori isolate Bali 03. Iron deficiency worsens the degree of gastric inflammation in BALB/c mice infected with H. pylori isolate Bali 03.

Features Of The Occurrence Of Helicobacter Pylori Infection With Gastroduodenal Pathology In Children

Despite the presence of studies devoted to the study of the bacterium Helicobacter pylori, its role in the pathogenesis of diseases such as chronic gastritis, gastric and duodenal ulcers, MALT lymphoma and gastric adenocarcinoma, the question of what determines the development of one or another clinical form of gastroduodenal pathology remains open. One of the factors with which the characteristics of clinical forms of Helicobacter pylori-associated diseases are usually associated is the genetic characteristics of the microorganism, which determines its virulence

Should All Patients With Stage IE Gastric Mucosa-Associated Lymphoid Tissue Lymphoma Receive Antibiotic Eradication Therapy for Helicobacter pylori?

H. pylori eradication therapy (HPE) can lead to tumor regression in H. pylori-positive (HPP) gastric mucosa-associated lymphoid tissue (MALT) lymphoma. However, some patients do not have detectable H. pylori (HP) infection (H. pylori-negative [HPN]) and the guidelines differ in their initial approach to HPN patients. The National Comprehensive Cancer Network (NCCN) recommends proceeding to radiation therapy, whereas European Society for Medical Oncology suggests HPE for every patient, even those who are HPN. To address this issue, we evaluated the effectiveness of HPE in limited-stage gastric MALT lymphoma. We retrospectively reviewed patients newly diagnosed with stage IE gastric MALT lymphoma between January 2002 and December 2022. The primary outcome was the complete remission (CR) rate defined as no macroscopic findings of lymphoma and negative gastric biopsy at the follow-up gastric endoscopy. Fifty-two patients were reviewed, and HP infection was detected in 19 (36.5%) patients-14 by immunostaining, three by serology, and one each by stool antigen and urea breath test. All 19 HPP and eight of the 33 HPN patients received HPE treatment. The CR rate was 63% (12/19) in HPP patients and 13% (1/8) in HPN patients (P = .033). After a median follow-up of 89.7 months, only two of the 12 HPP patients achieving CR have relapsed; the one HPN patient who received HPE remains in CR at 12+ months. For limited-stage HPP gastric MALT lymphoma, HPE is an effective and durable first-line treatment and should be used. For HPN patients, the CR rate with HPE is very low in our experience and is thus in support of the NCCN guideline.

Artificial intelligence-based differential diagnosis of orbital MALT lymphoma and IgG4 related ophthalmic disease using hematoxylin-eosin images.

To investigate the possibility of distinguishing between IgG4-related ophthalmic disease (IgG4-ROD) and orbital MALT lymphoma using artificial intelligence (AI) and hematoxylin-eosin (HE) images. After identifying a total of 127 patients from whom we were able to procure tissue blocks with IgG4-ROD and orbital MALT lymphoma, we performed histological and molecular genetic analyses, such as gene rearrangement. Subsequently, pathological HE images were collected from these patients followed by the cutting out of 10 different image patches from the HE image of each patient. A total of 970 image patches from the 97 patients were used to construct nine different models of deep learning, and the 300 image patches from the remaining 30 patients were used to evaluate the diagnostic performance of the models. Area under the curve (AUC) and accuracy (ACC) were used for the performance evaluation of the deep learning models. In addition, four ophthalmologists performed the binary classification between IgG4-ROD and orbital MALT lymphoma. EVA, which is a vision-centric foundation model to explore the limits of visual representation, was the best deep learning model among the nine models. The results of EVA were ACC = 73.3% and AUC = 0.807. The ACC of the four ophthalmologists ranged from 40 to 60%. It was possible to construct an AI software based on deep learning that was able to distinguish between IgG4-ROD and orbital MALT. This AI model may be useful as an initial screening tool to direct further ancillary investigations.

1037: Radiotherapy for mucosa-associated lymphoid tissue lymphoma: experience of a single institution

1037: Radiotherapy for mucosa-associated lymphoid tissue lymphoma: experience of a single institution

260: Volumetric modulated arc therapy for early-stage gastric mucosa-associated lymphoid tissue lymphoma

260: Volumetric modulated arc therapy for early-stage gastric mucosa-associated lymphoid tissue lymphoma

1198: Reduced-dose radiotherapy for stage IE gastric mucosa-associated lymphoid tissue lymphoma

1198: Reduced-dose radiotherapy for stage IE gastric mucosa-associated lymphoid tissue lymphoma

904: The efficacy of reduced-dose high-energy electron beam therapy in conjunctival MALT lymphoma

904: The efficacy of reduced-dose high-energy electron beam therapy in conjunctival MALT lymphoma

Camper’s and Scarpa’s fasciae: Anatomic landmark to differentiate between lymph node and abdominal wall involvement in a case of lymphoma

Cutaneous involvement of MALT lymphoma can either be primary or secondary, Secondary spread to the skin is considered disseminated extra-nodal disease involvement, which is classifies as a stage IV disease. We described a case of a 44-year-old woman with MALT lymphoma of the lacrimal glands with biopsy-proven lymphoma involvement in the subcutaneous tissue of the anterior abdominal wall. This involvement is manifested as multiple small discrete solid enhancing subcutaneous nodules. Although such findings on conventional CT scans are usually non-specific and may present in various systemic conditions, the knowledge of the patient’s prior clinical history should alert interpreting radiologists to be aware of cutaneous and subcutaneous involvement of lymphoma. This awareness can help plan the use of imaging-guided tissue biopsy for a minimally-invasive and safe acquisition of tissue specimen for histopathological diagnosis.

Study of individual domains contributing to MALT1 dimerization in BCL10-independent and dependent assembly

The CARMA-BCL10-MALT1 (CBM) signalosome functions as a pivotal supramolecular module, integrating diverse receptor-induced signaling pathways to regulate BCL10-dependent NF-kB activation in innate and adaptive immunity. Conversely, the API2-MALT1 fusion protein in t(11; 18)(q21; q21) MALT lymphoma constitutively induces BCL10-independent NF-kB activation. MALT1 dimer formation is indispensable for the requisite proteolytic activity and is critical for NF-kB activation regulation in both scenarios. However, the molecular assembly of MALT1 individual domains in CBM activation remains elusive. Here we report the crystal structure of the MALT1 death domain (DD) at a resolution of 2.1 Å, incorporating reconstructed residues in previously disordered loops 1 and 2. Additionally, we observe a conformational regulation element (CRE) regulating stem-helix formation in NLRPs pyrin (PYD) within the MALT1 DD structure. The structure reveals a stem-helix-mediated dimer further corroborated in solution. To elucidate how the BCL10 filament facilitates MALT1 dimerization, we reconstitute a BCL10-CARD-MALT1-DD-IG1-IG2 complex model. We propose a N+7 rule for BCL10-dependent MALT1 dimerization via the IG1-IG2 domain and for MALT1-dependent cleavage in trans. Biochemical data further indicates concentration-dependent dimerization of the MALT1 IG1-IG2 domain, facilitating MALT1 dimerization in BCL10-independent manner. Our findings provide a structural and biochemical foundation for understanding MALT1 dimeric mechanisms, shedding light on potential BCL10-independent MALT1 dimer formation and high-order BCL10-MALT1 assembly.

Non-Urothelial Malignancy in Urine Cytology

Non-urothelial malignancies of the urinary bladder are relatively rare and can be classified into primary and secondary malignancies. The histological subtypes of non-urothelial malignancy include non-urothelial carcinoma, sarcoma, lymphoma, and melanoma. The common types of primary non-urothelial carcinoma are pure squamous cell carcinoma, adenocarcinoma, and small cell carcinoma. Most primary sarcomas of the urinary bladder are leiomyosarcoma and rhabdomyosarcoma. Extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue origin and diffuse large B-cell lymphoma (DLBCL) are the common primary lymphomas of the urinary bladder. Most secondary tumors of the urinary bladder result from the direct extension of adjacent organs such as the colon/rectum, prostate, and cervix. Hematogenous metastasis from other primary cancers such as breast, stomach, lung, and skin (melanoma) are less common. The cytologic diagnosis of non-urothelial malignancies is challenging due to the significant cytomorphologic overlap with high-grade urothelial carcinoma, its divergent differentiation, and subtypes. Awareness of these diagnostic entities is helpful for accurate diagnosis, especially when encountering urine specimens with unusual cytomorphology.

A Case of Synchronous Primary Gastric and Duodenal Mucosa-Associated Lymphoid Tissue Lymphoma in Remission after Helicobacter pylori Eradication

A Case of Synchronous Primary Gastric and Duodenal Mucosa-Associated Lymphoid Tissue Lymphoma in Remission after <i>Helicobacter pylori</i> Eradication

P107 Real world clinical outcomes of patients with moderate-to-severe rheumatoid arthritis initiating upadacitinib in the United Kingdom: Interim analysis from a prospective observational cohort study (ENDEAVOUR)

Abstract Background/Aims Upadacitinib (UPA) 15 mg, a once-daily Janus kinase inhibitor (JAKi), is approved by NICE for treating adults with moderate-to-severe rheumatoid arthritis (RA) unresponsive to conventional Disease Modifying Antirheumatic Drugs (DMARDs). This study aims to evaluate UPA's real-world effectiveness in the UK. Methods ENDEAVOUR is a prospective observational cohort study which recruited 97 patients with moderate to severe RA whom the managing health care professional has decided to treat with UPA in line with marketing authorisation across 14 sites in the UK. Patient demographics, comorbidities, concomitant therapies and patient reported disease activity were collected at baseline, three months, and six months. Patient Reported Outcomes (PROs) were collected using an app hosted by UMotif. Primary objective was the proportion of patients achieving clinical remission (defined as DAS28&amp;lt;2.6) with upadacitinib at six months. The study is ongoing and therefore not all patients have completed study visits at three and six months yet however this interim analysis reports the baseline demographics of 77 patients whose data has been cleaned thus far and clinical outcomes and safety of UPA 47 adult patients at three months. Data are as observed. Results Data were available for 77 adults at baseline: 42% had moderate and 58% had severe RA based on DAS28-CRP scores. Patients' average age was 55 years, 64 (83%) were female, and most had at least one comorbidity, such as hypertension (16,21%) or asthma (12,16%). 32 (42%) had prior biologic therapy, with treatment failure the most commonly reported reason for switch (26/33, 79%). Of the 47 participants with three-month data, 18 (38%) achieved DAS28 remission. The proportion of patients with high disease activity decreased markedly from 45 (58%) at baseline to three (6%) at three months and moderate disease activity from 32 (42%) at baseline to 14 (30% at three months). Concurrent corticosteroid use declined, from 13 (17%) patients at baseline to just three (5%) by three months. The mean exposure time was six months (IQR five to seven). Nine patients (9%) stopped UPA during the study period, (5/9 due to adverse events, 4/9 due to patient or physician choice). One case of shingles occurred, with temporary treatment interruption. Reports of serious adverse events were low (4% patients of events reported) with cases of serious and opportunistic infections reported most frequently (three cases). There were no reports of MACE or venous thromboembolic events. 1 report of malignancy (extranodal marginal zone B-cell lymphoma) was considered unrelated to treatment. Conclusion This preliminary analysis of an observational study provides valuable insights into the real-world effectiveness of UPA for treatment of moderate to severe RA with similar early efficacy to that observed in clinical trials. 38% of patients achieved clinical remission by 3 months, and adverse events were infrequent. Disclosure J. Taylor: None. S. Srirangan: None. M. Bukhari: Honoraria; M.B has received honoraria for speaking and attended advisory boards with Bristol-Myers Squib, UCB celltech, Roche/Chugai, Pfizer, AbbVie, Merck, Mennarini, Sanofi-aventis, Eli-Lilly, Janssen, Amgen, Novartis and Gilead. Other; M.B. has been sponsored to attend regional, national and international meetings by UCB celltech, Roche/Chugai, Pfizer, Abbvie, Merck, Mennarini, Janssen, Bristol-Myers Squib, Novartis and Eli-lilly. S. Bilgrami: Other; S.B. has received professional and financial support from Eli Lilly, UCB &amp; Pfizer. M.K. Nisar: Other; M.K.N undertakes clinical trials and received support (including attendance at conferences, speaker fees and honoraria) from Roche, Chugai, MSD, AbbVie, Pfizer, BMS, Novartis, Celgene, UCB and, Lilly. S. McDonald: None. N. Goodson: None. A. Allard: Other; A.A. has received payment for services, sponsorship, funding, grants or worked on clinical trials for AbbVie, Eli Lilly, Pfizer, Novartis, BMS, Roche. A. Kinder: None. M. Green: None. L. Hunt: None. S. Raizada: Honoraria; S.R. has received honoraria from AbbVie. B. Kirkham: Consultancies; B.K. has received consultant/speaker fees from AbbVie, Eli Lilly, Galapagos, Janssen, Novartis, Pfizer and UCB. Grants/research support; B.K. has received research support from Eli Lilly and Novartis. E. Thomas: Other; E.T. has been on advisory boards for Janssen and Galapagos. R. Horsfall: None. S. Bartlett: None. R. Millward: None. V. Burton: None. J. Galloway: Consultancies; J.G. has received speaker/consultancy for AbbVie, Galapagos, Janssen, Lilly, Pfizer, UCB and Vifor. Grants/research support; He has received research grants for AbbVie, Galapagos, GSK, Janssen, Lilly, Pfizer.

Chinese expert consensus on the diagnosis and management of ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma (2023 edition)

Ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (OAML) is a common malignant tumor that affects the ocular adnexal region. The incidence of OAML is increasing due to the aging population. The tumor invades the ocular adnexal region, which can result in abnormal ocular appearance and function, thereby reducing the quality of life. Currently, there is no standardized diagnosis and management guideline for OAML. To enhance the standardization of diagnosis and management in OAML, a collaborative effort was undertaken by esteemed organizations in China. The Cellular Immune Therapy Committee of China Association for Promotion of Health Science and Technology, the Ocular Tumor Committee of Chinese Medical Doctor Association for Ophthalmologist Branch, the Imaging Medicine Branch of Chinese International Exchange and Promotion Association for Medical and Healthcare, the Tumor and Microecology Professional Committee of China Anti-cancer Association, and the Lymphoma Immunotherapy Committee of Beijing Cancer Prevention Society jointly convened a panel of experts to develop the inaugural "Chinese Expert Consensus on the Diagnosis and Management of ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma (2023 edition)"..