Type Of Lymphoma Research Articles

Syncope as a clinical presentation of cardiac lymphoma: a case report

Primary cardiac lymphoma, an extremely rare type of B-cell non-Hodgkin lymphoma, has an incidence of approximately 0.02-2% among malignant cardiac tumors. It is frequently associated with manifestations such as cardiac rhythm disturbances, refractory pericardial effusion and cardiac masses. Definitive diagnosis is achieved by biopsy, being essential to rule out hematogenous dissemination with PET-CT. The literature indicates a predominance of right chamber involvement and survival that varies significantly according to left ventricular involvement and the presence of arrhythmias. Therapeutic options are not well defined, but chemotherapy such as R-CHOP has shown efficacy, although the prognosis is generally poor due to complications such as disease progression, arrhythmias and sepsis. This case underscores the importance of considering cardiac lymphoma in patients with unexplained cardiac symptoms, highlighting the need for a multidisciplinary and specific diagnostic and therapeutic approach.

Primary breast lymphoma mimicking triple-negative breast cancer: a case report with clinical and pathological implications

BackgroundPrimary breast lymphoma (PBL) is a rare type of extranodal lymphoma, the diagnostic process for which presents significant challenges owing to an overlap in clinical and pathological features with those observed in triple-negative breast cancer (TNBC). However, the current literature reveals a paucity of information regarding the ramifications of potential diagnostic errors, particularly in the context of emergent therapeutic strategies for TNBC. Thus, we present a unique report of a case of PBL.Case presentationA 76-year-old female with no past medical or family history presented to the hospital with the chief complaint of a mass in the right breast. Two masses were palpated in the right breast: one 56 mm mass (No. 1) located at 10 o'clock, and a 21 mm large, elastic, hard mass (No. 2) at 4 o'clock. Needle biopsy was performed only on the larger 56 mm mass (No. 1). The results showed invasive carcinoma that was negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor-2. The preoperative diagnosis was right breast cancer (T3N0M0 Stage IIB) of the TNBC subtype. The patient refused the preoperative chemotherapy recommended by the treatment team; therefore, right breast mastectomy and sentinel lymph-node biopsy were performed instead. The histopathological diagnosis of the first mass was diffuse large B-cell lymphoma (DLBCL); that of the second mass (No. 2) was an invasive breast carcinoma of no special type. Postoperative treatment consisted of endocrine therapy (letrozole) for breast cancer, while the DLBCL was treated with chemotherapy and three courses of intrathecal chemotherapy. At the time of this report, the patient is still living, and neither tumor had recurred in the 2 years following surgery.ConclusionsOn rare occasions, PBL can preoperatively mimic TNBC. While this case did not lead to serious consequences, because surgery was eventually selected as the first therapy, clinicians should be aware that the diagnosis of PBL is challenging using only a core-needle biopsy and can often be misdiagnosed as TNBC.

Evaluation of immune sensor responses to a viral small noncoding RNA.

Gammaherpesviruses are widespread pathogens causing persistent infections linked to the development of numerous types of lymphomas in humans. During latency, most of the viral protein-coding genes are suppressed, facilitating evasion of adaptive immune recognition of protein antigens. In contrast, many noncoding RNA (ncRNA) molecules are expressed in infected cells and can regulate key cellular pathways while simultaneously evading adaptive immune recognition. To counteract this, many cells express internal pattern recognition receptors that can intrinsically sense ongoing infections and initiate cellular defenses. Murine gammaherpesvirus 68 (MHV68) is a valuable model to study in vivo aspects of gammaherpesvirus pathogenesis. The MHV68 ncRNA TMER4 (tRNA-miRNA-encoding RNA 4) promotes lymph node egress of infected B cells: in the absence of TMER4, MHV68-infected B cells accumulate in the lymph node in a manner similar to B cells activated through specific antigen encounter. We hypothesized that TMER4 may alter intrinsic immune activation. In research described here, we aimed to explore the immunomodulatory functions of TMER4 by evaluating its impact on signaling through the critical immune sensors Toll-like receptor 4 (TLR4), TLR3, TLR7, and retinoic acid-inducible gene I (RIG-I). To accomplish this, we developed a system to test noncoding RNAs using commercially available reporter cell lines. We optimized the experimental procedure to ensure ncRNA expression and to quantify immune sensory molecule induction or inhibition by the expressed ncRNA. Expression of TMER4 RNAs from plasmid constructs did not alter TLR or RIG-I signaling. This study provides a clear experimental framework that can be applied to test other small ncRNAs for their impact on various innate immune sensor proteins.

Fifteen Years of Non-Hodgkin Lymphoma in an Indonesian National Referral Hospital: Epidemiologic Trends and Diagnostic Challenges.

The global burden of lymphoma is substantial because of the increase in its incidence in recent decades. However, disease characteristics vary across different geographical locations. Numerous immunohistochemistry markers and molecular studies are essential to determine lymphoma diagnosis and prognosis. This poses significant challenges in developing countries with limited health care resources. This large-scale study assesses the frequency of non-Hodgkin lymphoma (NHL) in Indonesia over the past 15 years, analyses its clinicopathologic features, and predicts future trends. This retrospective study collected lymphoma patients diagnosed at the Department of Anatomical Pathology Dr. Cipto Mangunkusumo National Central General Hospital, Indonesia, from 2009 until 2023. All lymphoma diagnoses were confirmed by using ancillary tools classified as an enhanced lymphoma panel according to a resource-stratified guideline. We analyzed the clinicopathologic features of each NHL type and further applied the Autoregressive Integrated Moving Average model to predict future incidence trends. The study consisted of 7,368 NHL patients. Among these, B-cell lymphomas accounted for 90.6%, with diffuse large B-cell lymphoma being the most prevalent subtype (68.8%), followed by follicular lymphoma (8.8%) and marginal zone lymphoma (5.8%). Extranodal natural killer/T-cell lymphoma, nasal type, is the most common T-cell lymphoma found (26.3%). All types of lymphoma were found to be more common in males (57.7%). Extranodal involvement, particularly in the tonsil and upper respiratory tract, was frequently observed. Projection analysis indicates a steady increase in lymphoma patients in the future. This study highlights the distribution and burden of NHL in Indonesia over 15 years. The overall epidemiologic pattern of NHL in this study aligns with the results observed in other Asian countries. The rising incidence of lymphoma requires improved health care infrastructure and prevention strategies.

Sinonasal Plasmablastic Lymphoma: A Systematic Review

Abstract Objective Plasmablastic lymphoma (PBL) is a type of non-Hodgkin's B-cell lymphoma associated with human immunodeficiency virus and Epstein–Barr virus, commonly located in the oral cavity or gastrointestinal tract. Sinonasal involvement is rare, and there is no consensus on treatment. Data Sources Peer-reviewed published articles served as data sources. Review Methods A systematic review was conducted of the PubMed database for all cases of sinonasal PBL between 1978 and 2023 with the phrase “plasmablastic lymphoma.” Studies not written in English and that did not separate individual cases of sinonasal PBL from aggregated data were excluded. Age, sex, immune status, treatment, and outcomes were collected. Conclusion PBL is a rare malignancy in the sinonasal region usually treated with chemotherapy. It most commonly occurs in immunocompromised adults but has also been diagnosed in immunocompromised children and in immunocompetent adults. It is aggressive and has a poor prognosis. Implications for Practice PBL is a recently described entity with few cases of the sinonasal anatomic variant in the literature. Sinonasal PBL was most frequently treated with chemotherapy alone, closely followed by chemoradiation. The most common chemotherapy regimen utilized in the literature is cyclophosphamide, doxorubicin, oncovin/vincristine, and prednisone, which is also the most common chemotherapy regimen in nonsinonasal PBL. A second commonly used regimen is cyclophosphamide, vincristine/oncovin, doxorubicin/adriamycin, and dexamethasone. However, no treatment has emerged as superior to others with regard to survival. Further data are needed to better understand this rare disease.

CAR T-cell therapy combined with autologous hematopoietic cell transplantation in patients with refractory/relapsed Burkitt Lymphoma

Burkitt lymphoma (BL) is a highly aggressive type of non-Hodgkin lymphomas that have a high likelihood of relapse and are highly refractory to initial treatment. While high-intensity chemotherapy has improved the outcomes, many adult patients still experience treatment failure, and effective salvage therapies are limited. This study retrospectively analyzed the outcomes of 21 relapsed or refractory (R/R) adult BL patients treated with chimeric antigen receptor T-cell (CAR-T) therapy, combined or not with hematopoietic cell transplantation (HCT), across four Chinese hospitals. Patients were grouped based on treatment strategies: autologous HCT followed by CAR T-cell therapy (auto-HCT+CART group, n=8), and CAR T-cell therapy alone (CART group, n=13). The auto-HCT+CART group demonstrated superior outcomes, with an overall response rate (ORR) of 87.5% and significantly higher 1-year overall survival (OS) and progression-free survival (PFS) rates compared to the CART group (p=0.014 and p=0.045, respectively). These findings suggest that combining auto-HCT with CAR-T therapy may enhance long-term disease control in R/R BL patients. These encouraging results highlight the need for further prospective studies to validate our data.

Financial hardship and healthcare utilization among older cancer survivors with comorbidities.

349 Background: Financial burden among older cancer survivors (65y+) is understudied, particularly with regard to how comorbidities may affect financial burden and healthcare utilization despite Medicare eligibility. We assessed the relationship between financial hardship (FH), healthcare utilization, and comorbidities among cancer survivors. Methods: The University of Alabama at Birmingham tumor registry was used to identify patients diagnosed at ≥65y with lymphoma, breast, colorectal, prostate, or lung cancer types and received curative-intent treatment from 2000 to 2019. Patients were surveyed on FH, healthcare utilization, and comorbid conditions. FH was defined as having answered ‘Yes’ to experiencing material, psychological, or behavioral hardship in the past year. Visits to a primary care physician (PCP), oncologist, non-oncology specialist, or emergency department (ED) in the past 2 years were self-reported. A modified Common Terminology Criteria for Adverse Events (CTCAE) grading system captured 45 self-reported comorbidities. A CTCAE of 3 or 4 was considered severe. Multivariable models were used to determine the differences in risk of any FH between severe vs. non-severe CTCAE and to determine differences in healthcare utilization. All models were adjusted for age at survey completion, years from diagnosis to survey, sex, race, income, stage, distance travelled to medical center. Results: Among the 759 survivors included, median age was 77 (IQR=73-82), a majority identified as White (82%), half were female (53%) and a third reported annual income of less than 20,000 (32%). Breast (33%) was the most common cancer type followed by prostate (26%), lung (17%), colorectal (15%), and lymph (9%). Moderate or Severe comorbidities were reported by 46% of participants. FH was most commonly associated with coronary heart disease (18-47%), chronic obstructive pulmonary disease (20-57%), and myocardial infarction (25-60%) across all cancer types. Overall, patients with severe comorbidities had a higher risk of any FH (RR, 1.50; 95%CI, 1.15, 1.97), ED visits (IRR, 2.20; 95%CI, 1.22, 3.95), specialists (IRR, 1.52; 95%CI, 1.25, 1.87), and PCP (IRR 1.15; 95%CI, 1.01, 1.30). Those with severe comorbidities and financial hardship had higher utilization of PCP (p < 0.003), but not other types of utilization. Conclusions: Older patients with severe comorbidities were at increased risk of financial hardship while also utilizing healthcare facilities at a higher rate than those with fewer comorbidities. While those with severe comorbidities and financial hardship utilized their PCP more often, they did not utilize all healthcare facilities more than those with fewer comorbidities. Financial burden and high healthcare utilization remain after entering into cancer survivorship but is exacerbated by additional comorbid conditions.

Upfront Management of Blastoid Variant Mantle Cell Lymphoma: Making the Case for 2nd/3rd-Generation Bruton Tyrosine Kinase Inhibitor-Based Therapies.

Blastoid variant-mantle cell lymphoma (BV-MCL) represents an aggressive subset of patients with no established standard of care treatment approach. We performed a retrospective analysis of the clinical characteristics and outcomes of 17 de novo BV-MCL patients treated at our institution between May 2009 and September 2023. In addition, we reviewed the literature supporting 2nd/3rd generation Bruton's Tyrosine-kinase (BTKi)-based therapies for upfront management. The complete response rate to frontline and salvage therapy was 66.7% and 25%, respectively. Two-year overall survival (OS) was low at 62.5% (95% CI, 34.7%-81.1%). Variables associated with higher OS included receipt of consolidative HSCT (p = 0.017), TP53-wild type (p = 0.031), intermediate- versus high-risk Mantle Cell Lymphoma Prognostic Index score (p = 0.026), and achieving complete response with induction therapy (p = 0.027). Treatment outcomes with chemo-immunotherapy in BV-MCL patients are poor, especially among TP53-mutated patients. Recent findings describing positive outcomes with novel BTKi-based therapies are encouraging and should be considered in this high-risk population.

A novel anti-CD47 antibody with therapeutic potential for NK/T-cell lymphoma

ABSTRACT NK/T-cell lymphoma (NKTCL) is a rare type of non-Hodgkin lymphoma (NHL). Although L-asparaginase-based chemotherapy has significantly improved survival in early-stage patients, the prognosis is poor in advanced and relapsed or refractory patients. CD47 is a promising target for cancer immunotherapy. However, the expression of CD47 in NKTCL and the antitumor effect and mechanism of the anti-CD47 monoclonal antibody (mAb) AK117 in NKTCL remain unclear. Firstly, the expression level of CD47 protein in NKTCL cells was detected by immunoblot and flow cytometry. Secondly, in order to validate the role of CD47 downregulation in the proliferation, apoptosis, and cell cycle of NKTCL cells, we used shRNA transfection to knock down CD47 expression. We determined the effect of knocking down CD47 and the novel anti‐CD47 antibody AK117 on the phagocytosis of NKYS and YTS cells by M2 macrophages in vitro. Finally, we assessed the ability of AK117 to inhibit tumor growth in an NKTCL xenograft model in which YTS cells were engrafted in SCID mice. The results showed that CD47 is relatively highly expressed in NKTCL cells. CD47 knockdown in NKTCL promoted phagocytosis by M2 macrophages in an in vitro coculture assay. The study also demonstrated that anti-CD47 mAb AK117 promoted phagocytosis of NKTCL cells by M2 macrophages. In addition, in vivo experiments showed that the anti-CD47 mAb AK117 significantly inhibited the growth of subcutaneous xenograft tumors in SCID mice compared to the control antibody IgG. Our results indicate that targeting CD47 monoclonal antibodies is a potential therapeutic strategy for NKTCL.

Higher Microbial Abundance and Diversity in Bronchus-Associated Lymphoid Tissue (BALT) Lymphomas than in Non-Cancerous Lung Tissues.

It is well known that the majority of the extranodal marginal zone lymphomas of mucosa-associated lymphoid tissues (MALT lymphomas) are associated with microbiota, e.g., gastric MALT lymphoma with Helicobacter pylori. In general, they are very sensitive to low-dose radiotherapy and chemotherapeutic agents. The microbiota profile is not clearly elucidated in bronchus-associated lymphoid tissue (BALT) lymphoma, a rare type of MALT lymphoma in the lung. Thus, this study aimed to clarify the intratumor microbiome in BALT lymphoma using the third-generation NGS method. DNAs were extracted from 12 formalin-fixed paraffin-embedded (FFPE) tumor tissues obtained from BALT lymphoma patients diagnosed between 1990 and 2016. 16S rRNA gene was amplified by polymerase chain reaction. Amplicons were sequenced using a Nanopore platform. Next-generation sequencing analysis was performed to assess microbial profiles. For comparison, FFPE specimens from nine non-cancerous lung tissues were also analyzed. Specific bacterial families including Burkholderiaceae, Bacillaceae, and Microbacteriaceae were associated with BALT lymphoma by a linear discriminant analysis effect size approach. Although the number of specimens was limited, BALT lymphomas exhibited significantly higher microbial abundance and diversity with distinct microbial composition patterns and correlation networks than non-cancerous lung tissues. This study provides the first insight into intratumor microbiome in BALT lymphoma using the third-generation NGS method. A distinct microbial composition suggests the presence of a unique tumor microenvironment of BALT lymphoma.

A study of the relationship between circulating cytokines (interleukin-2 receptor and tumor necrosis factor receptor 2) and risk of B-cell non-hodgkin lymphoma.

Mature B-cell non-Hodgkin lymphoma (B-NHL) occurs due to uncontrolled B-lymphocyte clonal expansion. Cytokines can directly stimulate B-cell proliferation and prevent B-cell apoptosis. Dysregulation of cytokines may play an important role in the development of B-NHL by enhancing chromosomal translocation, which is the hallmark of B-NHL. Both interleukin 2 and tumor necrosis factor-α are proinflammatory cytokines and play important roles in the growth, differentiation, and apoptosis of B cells.We conducted a prospective case-control study applied to 50 patients with B-NHL at Kasr Al Aini Hospital, Cairo University, and 50 age- and sex-matched controls. Clinical, laboratory and imaging data were collected. In all patients and controls, sIL-2R and sTNF-R2 levels were measured by enzyme-linked immunosorbent assay (ELISA). The Spearman correlation test was used to evaluate the correlation between the studied cytokines and clinical, laboratory and imaging findings. Sensitivity analysis was conducted to detect the cutoff values of the studied cytokines.Serum levels of sIL-2R and sTNF-R2 were significantly higher in patients than in controls. Additionally, their levels were significantly higher in aggressive types and advanced stages of lymphoma. Also, the studied cytokines were significantly correlated with different clinical and laboratory parameters of lymphoma. The level of sIL-2R and sTNF-R2 were closely related to the type of lymphoma (P value ˂ 0.001 and 0.012, respectively), further it was also associated with the natural history of lymphoma (aggressive vs. indolent) (P value ˂0.001 and 0.04 respectively).We concluded that Pretreatment levels of sIL-2R and sTNF-R2 may play a role in the natural history and prognosis of lymphoma. They may be used as a prognostic factor for B-NHL patients and may also help with treatment decisions.

A Rare Coexistence of Turner Syndrome and Mycosis Fungoides: A Case Report.

Turner syndrome (TS) is the most common sex chromosome abnormality among females, characterised by short stature, hypergonadotropic hypogonadism, congenital heart anomalies, and an increased risk of autoimmune diseases. Although TS does not typically increase the absolute risk of malignancy, specific cancers, such as those affecting the nervous system and gastrointestinal tract and malignant melanoma, may occur more frequently. Mycosis fungoides (MF) is the most common type of primary cutaneous T-cell lymphoma, typically affecting adults but also seen in children and adolescents. We report an 11.2-year-old girl with TS presenting with substantial weight gain and short stature. Clinical examination revealed characteristic TS features and karyotype analysis confirmed mosaic TS. Following growth hormone (GH) therapy, the patient developed persistent, erythematous, itchy skin lesions diagnosed as CD4+ MF. GH therapy was discontinued, and topical steroids controlled the skin lesions effectively. MF in TS is rare and unexpected, especially in a child. The coexistence of these conditions suggests a potential link between TS and an increased risk of MF, possibly due to T-cell dysregulation or autoimmune processes. While the clinical course of MF is typically indolent, careful monitoring and annual dermatologic evaluations are recommended for TS patients, particularly when skin lesions are present. This is the first reported case of MF in a child with TS. This case emphasises the importance of carefully evaluating skin lesions in patients with TS and suggests considering MF as a differential diagnosis.

The causal effect of telomere length on the risk of malignant lymphoma: A Mendelian randomization study.

Telomere length (TL) has been implicated in the risk assessment of numerous cancers in observational studies. Nevertheless, the relationship between TL and malignant lymphoma remains unclear, displaying inconsistent patterns across different studies. A summary dataset for genome-wide association study of TL and malignant lymphoma was acquired from the OpenGWAS website. An extensive 2-sample Mendelian randomization (MR) analysis was performed, encompassing various methodologies such as MR-Egger, weighted median, weighted mode, simple mode, and the primary method of inverse-variance weighting (IVW). Sensitivity evaluations were performed using the Cochran Q test, MR-Egger regression, and leave-one-out analysis. The main method IVW revealed that TL substantially increased the risk of Hodgkin lymphoma (HL; odds ratio [OR] = 2.135; 95% confidence interval [CI] = 1.181-3.859; P = .012). Both the IVW and weighted median methods indicated statistical associations between genetically predicted TL and other types of non-HL (OR = 1.671, 95% CI = 1.009-2.768, P = .045; OR = 2.310, 95% CI = 1.033-5.169, P = .042). However, there was no association between TL and diffuse large B-cell lymphoma, follicular lymphoma, or mature T/natural Killer-cell lymphoma, and sensitivity analysis revealed no heterogeneity or horizontal pleiotropy, indicating that the causal effect was robust. Our study shows that TL plays different roles in different types of lymphomas. A longer TL significantly increases the risk of HL and other types of non-HL.

Deregulation and Shattering of Chromosomal Segments Containing Multiple Oncogenic Targets in the Pathogenesis of Diffuse Large B Cell Lymphoma, Not Otherwise Specified (DLBCL, NOS)

Diffuse large B cell lymphoma, not otherwise specified (DLBCL, NOS) is the most common type of non-Hodgkin lymphoma (NHL). Significant efforts have been focused on utilizing advanced genomic technologies to further subclassify DLBCL, NOS into clinically relevant subtypes. These efforts have led to the implementation of novel algorithms to support optimal risk-oriented therapy and improvement in the overall survival of DLBCL patients. The pathogenesis of DLBCL at the molecular level indicates copy number variation (CNV) as one of the major forms of genetic alterations in the somatic mutational landscape. Random deregulation that results in complex breaks of chromosomes and restructuring of shattered chromosomal segments is called chromothripsis. Gene expression changes influenced by chromothripsis have been reported in cancer and congenital diseases. This chaotic phenomenon results in complex CNV, gene fusions, and amplification and loss of tumor suppressor genes. We present herein a summary of the most clinically relevant genomic aberrations, with particular focus on copy number aberrations in a case that highlights DLBCL, NOS arising from relapsed Hodgkin lymphoma. The focus of our study was to understand the relationship between the clinical, morphological, and genomic abnormalities in DLBCL, NOS through multiple techniques for therapeutic considerations.

High-grade B-cell lymphoma with 11q aberration in the HIV setting: a clinicopathological study of 10 cases and literature review

High-grade B-cell lymphoma with 11q aberration (HGBL-11q) is a distinct lymphoma entity according to the 5th edition of the WHO classification of hematolymphoid tumors. It lacks MYC translocation but carries proximal gains and/or telomeric losses of chromosome 11q. This rare type of B-cell lymphoma is less frequently reported in people living with HIV (PLWH), and its exact frequency remains unclear. Our goal was to retrospectively analyze its frequency in a cohort of aggressive B-cell lymphomas in PLWH, including Burkitt lymphoma (BL, n = 35), diffuse large B-cell lymphoma (DLBCL, n = 48), high-grade B-cell lymphoma, not otherwise specified (HGBL-NOS, n = 13), which was diagnosed as AIDS-related lymphoma (ARL) at our institution. In total, 10/96 (10.4%) cases harbored the typical 11q aberration pattern, predominantly those that had been classified as BL (6/35, 17.1%), DLBCL (2/48, 4.2%), and HGBL, NOS (2/13, 15.4%). We also evaluated 7 cases of AIDS-related HGBL-11q (AR-HGBL-11q) reported in the literature. The median age of our cohort was 35 years, and all the patients were male. Most cases (70%) had a history of HIV infection for over 1 year, and all were involved in lymph nodes (100%), frequently involved extranodal sites (60%), and Ann Arbor stage III/IV. In histomorphology, the cases exhibited diverse cytological features, reminiscent of BL (6 cases), DLBCL (2 cases), and HGBL (2 cases). A comparison of the combined cohort of 17 AR-HGBL-11q cases with 11 ARL cases that lacked both MYC rearrangement and 11q aberration at our institution showed that HGBL-11q cases were characterized by strikingly coarse apoptotic debris (P < 0.001), background rich in eosinophils (P = 0.002), higher expression of the germinal centre marker LMO2 (P = 0.080), lower expression of MUM1 (P = 0.004), BCL2 (P = 0.007), and LEF1 (P = 0.080), and lower positivity for EBER in situ hybridisation (P = 0.027). Notably, one case in our series was EBV-positive, a finding not previously reported in the literature. Furthermore, comparing the prognosis between these two groups, AR-HGBL-11q showed a relatively favorable prognosis (P = 0.15), although the difference was not statistically significant. We analyzed this rare lymphoma entity in the HIV setting and highlighted the importance of integrating histomorphological and immunophenotypic features in its diagnosis and classification.

Епідеміологічні дані, етіологія, патогенез, фактори ризику, клінічні прояви, сучасні методи діагностики і лікування, прогнозування перебігу Неходжкінських лімфом (огляд літератури)

Non-Hodgkin lymphoma (NHL) is a group of malignant diseases characterized by clonal proliferation of lymphoid cells corresponding to different stages of differentiation of normal B- and T- lymphocytes or natural killer (NK) cells. NHL is histopathologically and clinically diverse and divided into two groups: B-cell lymphomas and T-cell lymphomas, and subtypes: indolent (slow) and aggressive, classic and extranodal. The etiology of NHL is environmental, infectious, immunological, iatrogenic and genetic factors. The pathogenesis of NHL consists in: 1) translocations of oncogenes; 2) cytogenetic aberrations and mutations of tumor suppressor genes. Clinical manifestations of NHL: 1) lymphadenopathy; 2) symptoms of the presence of an extranodal tumor (abdominal pain; jaundice; shortness of breath and/or pleural effusion; bleeding, symptoms of gastrointestinal obstruction; symptoms associated with infiltration by lymphoma cells of the skin, thyroid gland, salivary glands, etc.; neurological symptoms of central and/or peripheral origin); 3) symptoms of bone marrow infiltration; 4) general symptoms (fever, night sweats, weight loss, fatigue, loss of appetite). Diagnosis of NHL involves: 1) auxiliary research (histological, immunohistochemical, cytogenetic and molecular, use of more specific monoclonal antibodies, immunophenotypic diagnosis, gene sequencing); 2) diagnostic tactics (estimation of stage and determination of prognostic factors according to IPI); 3) differential diagnosis (covers other causes of symptoms). The choice of treatment program for NHL depends on the type of lymphoma and the patient's condition. The main methods of treatment: wait and watch; monotherapy with alkylating drugs; RT; combined РCT; combined РCT with the use of purine analogs; therapy with monoclonal antibodies; PCT+monoclonal antibodies; HDCT+THSC. Survival prognosis for NHL depends on the origin of the tumor and its stage.

500. PRIMARY ESOPHAGEAL LYMPHOMA: UNDERSTANDING PROGNOSTIC FACTORS AND OUTCOMES

Abstract Background Primary Esophageal Lymphoma (PEL) represents a rare subtype of gastrointestinal lymphomas, posing unique diagnostic and therapeutic challenges. Given its rarity, comprehensive insights into its prognostic factors and outcomes remain limited. This study examines the epidemiology, treatment modalities, and outcomes of PEL, aiming to identify significant prognostic factors that influence patient survival. Methods A systematic review of the literature was conducted across PubMed/Medline and Scopus databases, focusing on cases of adult PEL. Data extraction included demographic information, treatment modality, and patient outcomes. Survival rates were calculated, and prognostic factors were analyzed through unifactorial analysis. Results Out of 56 studies, 69 cases of PEL were analyzed, with a male-to-female ratio of 2.8:1 and mean age of 55.5 years. Patients were predominantly treated with chemotherapy (52.1%) while 20% underwent surgical treatment. The mean survival reported was 49.3 months with one-, three-, and five-year survival rates of 84.5%, 72.3%, and 28.9%, respectively, exhibiting a less favorable prognosis compared to common lymphoma types. Survival analysis revealed that local lymphadenopathy had a significant impact on prognosis (p=0.008), while gender, age, treatment approach, and type of lymphoma were not significant determinants of survival. Immunocompromised status showed a marginally significant effect on survival (p= 0.053). Discussion The study highlights the less favorable prognosis of PEL compared to more common lymphomas, emphasizing the importance of recognizing patients with unfavorable prognostic factors such as local lymphadenopathy and immunocompromised status. The findings advocate for the establishment of an international patient registry to better understand and manage PEL, aiming to standardize diagnostic criteria and treatment strategies.

Predictive value of prognostic nutritional index as prognostic biomarkers in patients with lymphoma: a systematic review and meta-analysis.

Several research have indicated the significant potential of the Prognostic Nutritional Index (PNI) as a prognostic biomarker in lymphoma patients. However, there is some inconsistency in the findings of a few studies. Hence, to offer a thorough evaluation of the predictive significance of PNI in lymphoma patients, we performed a meta-analysis to examine the prognostic value of PNI for survival outcomes in lymphoma patients. We conducted a comprehensive search for pertinent works published up until December 2023 in databases such as PubMed, EMBASE, Cochrane Library, and Web of Science. We obtained hazard ratio (HR) data related to survival outcomes and computed aggregated HRs with their corresponding 95% confidence intervals (CIs) to evaluate the correlation between PNI and both overall survival (OS) and progression-free survival (PFS) in lymphoma patients. By analyzing data from 1260 patients in 28 studies, we found that PNI levels were associated with prognosis in lymphoma patients. High PNI levels predicted that patients had longer OS (HR: 0.46, 95% CI 0.37-0.58, P < 0.05) and better PFS (HR: 0.56, 95% CI 0.45-0.70, P < 0.05). Subgroup analyses showed that the predictive ability of PNI for patient prognosis may differ depending on the type of lymphoma. In addition, we found that the critical PNI value had greater predictive potential at 40-45 and above 45. Our study suggests a strong association between PNI and prognostic outcomes in lymphoma patients, indicating that PNI holds substantial prognostic value in this population.

Ocular adnexal lymphoma - a retrospective study and review of the literature.

To review the characteristics of all Slovenian patients with ocular adnexal lymphoma (OAL) in the period of 24 years with the aim of evaluating demographic data, lymphoma location and type, disease stage, treatment modality, local control rate and survival rate. All patients with histologically diagnosed OAL in the main tertiary centre of Slovenia, Eye Hospital, University Medical Centre Ljubljana, who were treated at Institute of Oncology Ljubljana were included in the study. Patients' data were collected from October 1995 through April 2019. Seventy-four patients were included in the study having a median age of 68 years at diagnosis. The majority of lymphomas were of B-cell origin (98.6%). The most frequent type was the extranodal marginal zone B-cell lymphoma (MALT) (71.6%). Orbital lymphomas were diagnosed in 56 cases (75.7%) and conjunctival in 18 cases (24.3%). Ocular manifestation was the first sign of the disease in 78.4% of patients and in 67.6% of patients ocular adnexa were the only disease location. Fifty-one patients (68.9%) were treated with radiotherapy, 7 patients (9.4%) with systemic treatment, 5 patients (6.8%) with combined radiotherapy and systemic treatment and in 11 patients, biopsy and active surveillance strategy was applied (14.9%). Local control of the disease was achieved in 96.6% of treated patients. Median overall survival of the whole study group has not been reached yet. Five-year overall survival rate was 80.1% (95% CI 68.1% - 88.5%) and 5-year lymphoma specific survival rate was 87.2% (95% CI 83.2%-91.2%). OALs comprise a group of heterogeneous diseases with variable outcomes depending predominately on the patient's age and lymphoma type, with low grade lymphomas carrying good prognosis even in elderly patients.

Standardization of CD30 immunohistochemistry staining among three automated immunostaining platforms.

The identification of CD30 expression by immunohistochemistry is essential for the treatment of lymphomas using an antibody-drug conjugate targeting CD30. However, no standardized protocol for CD30 staining has been available. In this study, we compared three common automated immunostaining platforms {Bond III (B III), Dako Omnis (DO) and Ventana BenchMark ULTRA (VBMU)}. A primary antibody for CD30, the Ber-H2 clone, was diluted 50- to 400-fold for B III and DO, and ready-to-use antibody was used for VBMU. An enhancement step using a linker was introduced in all protocols. First, several candidate dilutions were selected for each platform by staining six cases. These candidate conditions were then confirmed with 60 cases of various types of peripheral T-cell lymphomas (PTCLs). The concordance rates of CD30 expression among platforms differed depending on cutoff values and antibody dilutions, except for anaplastic large cell lymphoma. The concordance rates among three platforms in the evaluation of "positive" or "negative" were 100% and 97% when the cutoff values were 1% and 10% respectively, if using 400-diluted antibody in B III and 100-diluted antibody in DO. This study demonstrated the feasibility of equalizing CD30 staining of PTCLs among different platforms by adjusting protocols.