Extranodal Involvement Research Articles

Whole-body magnetic resonance imaging provides accurate staging of diffuse large B-cell lymphoma, but is less preferred by patients.

Whole-body magnetic resonance imaging (wbMRI) allows general assessment of systemic cancers including lymphomas without radiation burden. To evaluate the diagnostic performance of wbMRI in the staging of diffuse large B-cell lymphoma (DLBCL), determine the value of individual MRI sequences, and assess patients' concerns with wbMRI. In this single-center prospective study, adult patients newly diagnosed with systemic DLBCL underwent wbMRI on a 3T scanner [diffusion weighted images with background suppression (DWIBS), T2, short tau inversion recovery (STIR), contrast-enhanced T1] and fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) (reference standard). The involvement of 12 nodal regions and extranodal sites was evaluated on wbMRI and PET/CT. The utility of wbMRI sequences was rated on a five-point scale (0 = not useful, 4 = very useful). Patients received a questionnaire regarding wbMRI. Of 60 eligible patients, 14 (23%) were enrolled and completed the study. The sensitivity of wbMRI in the nodal involvement (182 nodal sites) was 0.84, with 0.99 specificity, positive predictive value of 0.96, negative predictive value of 0.97, and 0.97 accuracy. PET/CT and wbMRI were concordant both in extranodal involvement (13 instances) and staging (κ = 1.0). The mean scores of the utility of MRI sequences were 3.71 ± 0.73 for DWIBS, 2.64 ± 0.84 for T1, 2.14 ± 0.77 for STIR, and 1.29 ± 0.73 for T2 (P < 0.0001). Patients were mostly concerned about the enclosed environment and duration of the MRI examination (27% of patients). The wbMRI exhibited excellent sensitivity and specificity in staging DLBCL. DWIBS and contrast-enhanced T1 were rated as the most useful sequences. Patients were less willing to undergo wbMRI as a second examination parallel to PET/CT, especially owing to the long duration and the enclosed environment.

P-13 EXTRANODAL LYMPHOMA WITH ENDOCRINE ORGAN INVOLVEMENT: CASE REPORTS

Abstract Introduction The incidence of extranodal lymphomas has increased in the last decade, most commonly occurring in the gastrointestinal tract, head and neck, skin/soft tissue and central nervous system. We report two cases of extranodal lymphoma in the thyroid and adrenal gland. Clinical Cases A 61-year-old male patient diagnosed with Small Lymphocytic Lymphoma (CLL) was referred to us after his follow-up at the Hematology Clinic. Thyroid ultrasonography (USG) revealed a nodular ovoid lesion, that has a semisolid structure of 36x12 mm in size, with a cystic component in the center, located at the right lobe - isthmus junction, and with increased vascularity. Laboratory tests were as in Table 1. It was learned that the patient had received chemotherapy for CLL in 2009 and was in remission, and there was no family history of thyroid cancer. Since the fine needle aspiration biopsy (FNAB) was suspicious for lymphoid neoplasia, a tru-cut biopsy was performed. The flow cytometry result was found to be compatible with B-cell clonality, and the pathology result revealed mature B-cell neoplasia infiltration. Immunohistochemically, dense plasma cells were observed with CD138. Thyroidectomy was recommended to the patient, but he refused and was followed up. A 73-year-old female patient was referred to our clinic with the suspicion of adrenal insufficiency due to ongoing nausea, vomiting, and hypotension symptoms. Laboratory tests at presentation were as shown in Table 2. The patient was started on 80 mg/day methylprednisolone, which was thought to be acute adrenal insufficiency biochemically and clinically, and since hyperkalemia persisted, 0.05 mg/day fludrocortisone was added to the treatment. It was learned that the patient had been examined for abdominal pain for 6 months, and 15 mm nodular lesions were observed in both adrenal glands in the abdominal CT, but no further examination was performed. In adrenal CT, lesions measuring 41x30 mm on the right and 30x22 mm on the left at the body-medial crus level were observed in both adrenal glands, with a density higher than expected for adenoma (Figure 1). The findings were evaluated as significant in favor of nonadenomatous lesions, and due to progression in lesion sizes and high suspicion of malignancy, pheochromocytoma was excluded by checking urine catecholamines from the lesion in the right adrenal gland, and a tru-cut biopsy was performed. As a result of the pathology, the patient was diagnosed with high-grade B-cell neoplasia and hematological treatment was started. Conclusion Extranodal lymphoma involvement has been described with multiple different presentations in every tissue of the body, even within an organ system. Therefore, it should be included in the differential diagnosis of mass lesions. Tissue biopsy and immunohistochemistry are the most important methods for determining the specific subtype and cell origin.Figure 1:CT images of patientLesions in bilateral adrenal glands Table 1:Laboratory workup*Thyroid Stimulating Hormone. Table 2:Laboratory workup*Blood Urea Nitrogen,C-reactive Protein, Adrenocorticotropic hormone, Thyroid Stimulating Hormone

Clinical, Pathologic, and Molecular spectrum of Angioinmmunoblastic T-cell Lymphoma Cutaneous Lesions: Clinical, Pathologic, and Molecular Analysis.

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive malignancy that frequently presents with extranodal involvement. Cutaneous tropism is clinically and histopathologically variable, which may pose a diagnostic challenge. We conducted a retrospective analysis of 40 samples of 20 cases of cutaneous AITL, focusing on the clinicopathologic and molecular correlations between skin and lymph node (LN) samples. In all cases, cutaneous involvement was concurrent with or followed the diagnosis of nodal AITL, with no cases preceding systemic involvement. Clinically, cutaneous AITL presented in 2 main forms: an evanescent rash and persistent lesions, with histopathology revealing diverse infiltration patterns, including perivascular, nodular, granulomatous, panniculitic, vasculitis, and epidermotropic. Clinical presentation and histologic patterns tend to correlate. Histopathologically, plasma cells were present in 15/22 skin samples, 5 of them being kappa-light restricted but polytypic in corresponding LNs. Epstein-Barr virus+ B cells were present in 10 cutaneous lesions and were already present in corresponding LNs. Molecular studies found correlations in all but one case between LN and skin, particularly in the presence of RHOA and TET2 mutations, which were identified in 8 of 12 cases. Molecular analysis was also informative in 4 cases with low levels of infiltration. The study also highlighted unique cases with distinct clinical and histopathologic patterns coexisting in the same patient over time. One case exhibited simultaneous granulomatous and epidermotropic patterns in different skin lesions. Four cases of cutaneous B-cell lymphomas associated with AITL were identified. Our study underscores the importance of integrating clinical, histopathologic, and molecular data to accurately diagnose cutaneous AITL.

Diffuse Large B-Cell Lymphoma/High Grade B-Cell Lymphoma with MYC and BCL6 Rearrangements: a study of 60 Cases.

Classification of cases of diffuse large B-cell lymphoma (DLBCL)/high-grade B-cell lymphoma (HGBL) with MYC and BCL6 rearrangements, also known as BCL6 double hit lymphoma (DHL), is controversial. We assessed 60 cases of BCL6-DHL and compared this cohort to 224 cases of DHL with MYC and BCL2 rearrangements (BCL2-DHL) and 217 cases of DLBCL not otherwise specified. Compared with the DLBCL cohort, BCL6-DHL patients had more aggressive clinical features such as frequent extranodal involvement, high-stage disease, high IPI score and elevated serum lactate dehydrogenase level (p <0.01 for all). Compared with the BCL2-DHL cohort, BCL6-DHL patients had similarly aggressive clinical features but a lower frequency of germinal center B-cell (GCB) immunophenotype and MYC and BCL2 double expression. Patients with BCL6-DHL showed an overall survival (OS) intermediate between patients with DLBCL and BCL2-DHL. Following induction with R-CHOP chemotherapy, BCL6-DHL patients demonstrated a poor OS similar to BCL2-DHL patients and worse than that of DLBCL patients (p = 0.024). However, among patients who received R-EPOCH, there was no significant difference in OS among the three groups (p = 0.146). Gene expression profiling showed that 60% of BCL6-DHL cases had a double hit (DH)-like signature compared with 10% of DLBCL-GCB and 93% of BCL2-DHL. The DH-like signature in BCL6-DHL cases was associated with a GCB immunophenotype. Based on these data, we suggest that BCL6-DHL cases are clinically more aggressive than DLBCL and patients may benefit from a more aggressive therapy than R-CHOP. The data also suggest that BCL6-DHL as currently defined, is heterogeneous and that neoplasms with a GCB immunophenotype are more likely to have DH-like signature and behave more aggressively. Lastly, we suggest that BCL6-DHL cases deserve to be recognized separately in a lymphoma classification to facilitate further understanding of these neoplasms and for optimal patient management.

Influence of Organ-Specific Extranodal Involvement on Survival Outcomes in Stage IV Diffuse Large B-Cell Lymphoma.

The prognostic significance of extranodal sites in stage IV diffuse large B-cell lymphoma (DLBCL) remains uncertain, making it challenging to select appropriate treatment strategies for individual patients. In this study, we aimed to evaluate the influence of different extranodal sites on prognosis in young patients with stage IV DLBCL who achieved complete remission (CR) following initial chemo-immunotherapy and to explore the potential of autologous hematopoietic stem cell transplantation (ASCT) as a consolidation treatment for specific patient subgroups. We retrospectively reviewed data from 119 patients with DLBCL aged < 60 years who achieved CR after chemo-immunotherapy between 2008 and 2020. Patient survival rates were analyzed in correlation with different extranodal sites using univariate and multivariate models. Additionally, we assessed the effect of ASCT on 5-year progression-free survival (PFS) and overall survival (OS) in patients with different extranodal sites involved. A retrospective bicenter study. Univariate analysis revealed a significant decrease in survival rates in patients with a Deauville score of 3 and those with extranodal DLBCL affecting the spleen, bone marrow, nasosinus, and liver. In multivariate analysis, only nasosinusal involvement remained a significant predictor of reduced OS. Patients with spleen involvement benefited significantly from ASCT in terms of 5-year PFS and OS, whereas those with nasosinusal involvement did not demonstrate any survival advantage with ASCT. Our findings highlight the influence of specific extranodal sites on the prognosis of patients with stage IV DLBCL. The data indicate a clear need for precise patient stratification based on extranodal involvement for more effective treatment planning. Notably, patients with spleen involvement appear to benefit from ASCT, suggesting that this strategy could be useful in this subgroup. Further prospective studies are needed to confirm and incorporate these findings into clinical practice.

Primary head and neck lymphoid neoplasms in adolescents and young adults: demographics, distribution and survival outcomes.

Primary head and neck lymphoid neoplasms(PHNLN) are described as a series of lymphoid system-derived neoplasms which originally arising from head and neck region. Our study is aimed to present a panoramic view of PHNLN among adolescent and young adult(AYA) patients aged from 15 to 39 years-old. The individual patient information was obtained from Surveillance, Epidemiology and End Results(SEER) database. Male patients outnumbered female patients in most pathological subtypes, with noticeable male predilection observed in Burkitt lymphoma and diffuse large B-cell lymphoma(DLBCL). Classical Hodgkin lymphoma(CHL) accounted for 92.23% of Hodgkin lymphoma. Mature B-cell neoplasms constituted the majority of non-Hodgkin lymphoma(NHL). DLBCL was the most common pathological subtype, followed by follicular lymphoma(FL). Tonsil, salivary glands (especially parotid gland) and nasal cavity were the most three frequent extranodal organs involved. Patients with extranodal involvement exhibited worse prognosis compared to those with lymph node confinement. Patients who suffered from precursor NHL and mature T/NK-cell NHL exhibited prolonged disease-specific survival compared to those with HL, PCN and mature B-cell NHL. AYA patients with absence of other SPM showed dramatic lower risk of death than those with occurrence of SPM. Patients with HL had a favourable survival advantage over those with mature B-cell NHL. Patients with precursor NHL and mature T/NK-cell NHL were at remarkable higher risk of death than those with mature B-cell NHL. Our study elucidated the demographics, distribution of anatomic sites and pathological subtypes, and survival outcomes of PHNLN among AYA population, enhancing comprehension of this rare sort of cancer entities.

Efficacy and Safety of Polatuzumab Vedotin Plus Rituximab, Cyclophosphamide, Doxorubicin and Prednisone for Previously Untreated Diffuse Large B-Cell Lymphoma: A Real-World, Multi-Center, Retrospective Cohort Study.

Polatuzumab vedotin plus R-CHP (Pola-R-CHP) is approved as a new standard first-line therapy for diffuse large B-cell lymphoma (DLBCL) based on the POLARIX trial. However, real-world data on its efficacy and safety in unselected patients is lacking. We conducted a retrospective cohort study to evaluate Pola-R-CHP versus R-CHOP outcomes in routine clinical practice in China. This is a multi-institutional retrospective cohort study and included all consecutive patients that received at least one dose of polatuzumab vedotin up until February 2024. A total of 600 eligible patients from 6 centers were identified, 131 receiving Pola-R-CHP and 469 R-CHOP. After 1:2 propensity score matching, 128 pairs were obtained for further survival and prognosis analysis. With a median follow-up of 12.8months, 12-month progression-free survival (PFS) was numerically higher with Pola-R-CHP versus R-CHOP (90.3% vs. 84.1%, p=0.18). Benefits were consistently observed across molecular subgroups, especially advanced stage, ECOG ≥ 2, extranodal involvement ≥ 2 and non-GCB group. The complete response rate of the Pola-R-CHP group was higher than that of the RCHOP group (86.8% vs. 79.7%; p=0.09), but there was no statistical difference. Safety profiles were comparable, with no new concerns. Among 128 patients treated with Pola-R-CHP, 96 underwent gene sequencing analysis: MCD (25.0%), EZB (13.5%), combined subtype (12.5%), ST2 (9.4%), and other/unclassifiable subtype (30.2%). The most common mutations (> 25% of cases) were PIM1, TP53, BCL-6, KMT2D, SOCS1, BCL-2. Genetic testing results show the correlation between genotyping, gene mutations in PIM1/TP53 and therapeutic efficacy. This large real-world study supports Pola-R-CHP as an effective frontline option for DLBCL, with sustained efficacy versus R-CHOP observed in unselected populations. While 12-month PFS failed to reach statistical significance, subgroup analyses favor Pola-R-CHP. Further research with a wider population, longer follow-up, and screening of advantageous groups are warranted.

An Isolated Cutaneous Relapse in a Known Case of Systemic ALK-Positive Anaplastic Large Cell Lymphoma: A Rare Case Report with Review of Literature

AbstractAmong non-Hodgkin's lymphomas (NHLs), anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) is a relatively uncommon subtype that accounts for 3% of all adult NHLs. It typically affects young males, with a prevalence of three to one. Most cases present with nodal disease at the time of presentation. An extranodal involvement is seen in 60% of cases and skin involvement is seen in only 8 to 21% of cases. Cutaneous involvement in ALCL can manifest as primary cutaneous ALCL or secondary to systemic ALCL, and while CD30 positivity is common to both, ALK is not expressed by the former. A secondary skin involvement is usually associated with a poorer prognosis.We report a rare case of an isolated cutaneous relapse of systemic ALK-positive ALCL in a 62-year-old woman following the second cycle of chemotherapy. The acute febrile, widespread papulonodular eruption clinically resembled mycosis fungoides and lymphomatoid papulosis. With the introduction of oral crizotinib, a drastic improvement in the skin lesions and an exceptional response on positron emission tomography-computed tomography were noted.

Destombes-Rosai-Dorfman disease: A case series of nodular and extra nodular involvement

Introduction and importanceRosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder characterized by painless lymph node enlargement and extranodal involvement. This disease often presents diagnostic challenges due to its variable clinical manifestations and the need for histologic confirmation. Case series presentationThis case series examines five patients diagnosed with Rosai-Dorfman disease who presented with a variety of clinical manifestations. Patients ranged in age from 21 to 72 years and presented with a variety of symptoms including cervical lymphadenopathy, mediastinal adenopathies, thyroid nodules, and nasal obstruction. Diagnoses were confirmed by histologic examination and immunostaining, primarily with anti-PS100 antibody. Treatments included surgery, corticosteroids, and laser resection, with most cases showing favorable outcomes and no recurrence at follow-ups ranging from one to five years. Clinical discussionThe diverse presentations of RDD in this case series underscore the importance of considering this entity in the differential diagnosis of lymphadenopathy and other mass lesions. Histologic examination and immunostaining are critical for accurate diagnosis. ConclusionThis case series aims to highlight the diverse presentations, diagnostic approaches, and treatment outcomes of patients with Rosai-Dorfman disease and to contribute to a better understanding of this rare condition.

A rare presentation of isolated intraosseous Rosai-Dorfman disease of the tibia – The role of whole-body magnetic resonance imaging in management

Rosai–Dorfman disease (RDD) is a rare non-Langerhans histiocytic disorder, usually presenting with enlarged cervical lymph nodes. Extranodal involvement is quite common and synchronously involves the nasal cavity, paranasal sinuses, skin, orbit, central nervous system, and rarely bones. Isolated bone involvement is rare and can present with local symptoms such as pain, swelling, restriction of activity, and occasionally pathological fracture. The most common sites of osseous RDD are the cranium, facial bones, and tibia, in decreasing order of frequency. Imaging findings of primary osseous RDD can be variable and there are no key features to diagnose this entity with certainty. Hence, the primary role of imaging is to plan the management and post-treatment follow-up. Here, we present a case of primary osseous RDD involving tibial diaphysis and emphasize the importance of whole-body magnetic resonance imaging in ruling out systemic involvement.

Can Circulating Tumor DNA Improve the Predictive Value of Interim Response in Patients with Advanced Hodgkin Lymphoma and PET-2 DS3?

Background: Analysis of 441 patients with classical HL in advanced stages from the Czech Hodgkin Lymphoma Registry treated with 6 or 4 cycles of eBEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone in escalated doses) according to the interim PET-2 has shown that within patients that received 4 cycles of eBEACOPP, there is a trend for a higher 5-year progression-free survival (PFS) in patients with PET-2 Deauville score (DS) 1-2 in comparison to patients with PET-2 DS3 (91% [95% CI 84-99] vs. 78% [95% CI 64-95], p=0.061), accepted for ISHL13. To better stratify patients with PET-2 DS3 into 4 or 6 cycles of eBEACOPP, we analyzed circulating tumor DNA (ctDNA) within the ongoing trial NCT06263530 and correlated it with interim PET-2 results. Methods: To analyze ctDNA levels and its dynamics, we collected peripheral blood from 67 patients with classical HL in advanced stages III/IV, including clinical stages IIB with massive mediastinal tumor and/or extranodal involvement. 60 patients received eBEACOPP (30 patients 4 cycles and 30 patients 6 cycles), 4 patients received combination of eBEACOPP (4 cycles) and ABVD (2 cycles), and only 3 patients received 6 cycles of ABVD. Blood samples were collected at diagnosis, after two cycles of chemotherapy, and at the end of treatment (EoT). Following cell free DNA extraction, levels of ctDNA were determined using a custom panel of 538 genes frequently altered in lymphomas utilizing next generation sequencing-based CAPP-Seq (CAncer Personalized Profiling by deep Sequencing) approach. Plasma ctDNA levels were consequently correlated with PET results at the same timepoints. Results: Out of 67 patients (all white race, male gender 39 patients, median age at diagnosis 33 with range 19 - 70 years) 56 achieved PET-2 metabolic complete remission - CMR (30 patients DS1-2 and 26 patients DS3) and 11 patients achieved partial remission - PR (DS4-5). At the end of chemotherapy, the number of CMR increased to 61, 5 patients were in PR, and unknown response was achieved in 1 patient. Overall, 9 patients relapsed/progressed after the first-line treatment (with interim PET-2: DS2 in 3 patients, DS3 in 2 patients, and DS4-5 in 4 patients). Preliminary analysis suggests that ctDNA might improve PET-2 based disease status evaluation and patient stratification. Correlation of ctDNA with interim PET-2 and EoT PET will be presented. Conclusion: ctDNA is a promising sensitive tool to better stratify treatment of patients with Hodgkin lymphoma. This work was supported by grants AZV NU22-03-00182 from the Ministry of Health of the Czech Republic, MH CZ DRO (FNOl_00098892, VFN00064165), National Institute for Cancer Research (EXCELES - LX22NPO5102), Cooperatio Program (research areas Oncology and Haematology and Biology), and SVV 260637.

Extranodal Mantle Cell Lymphoma Presented as Bilateral Adrenal Masses on 18 F-FDG PET/CT and 68 Ga-DOTATATE PET/CT.

Mantle cell lymphoma (MCL) is a rare type of B-cell non-Hodgkin lymphoma that commonly presents with constitutional symptoms, lymphadenopathy, and laboratory abnormalities. Although less common, extranodal involvement of MCL may be seen at initial presentation and most commonly occurs in the spleen, liver, bone marrow, and gastrointestinal tract. Adrenal involvement in MCL is extremely rare, and only a few cases have been reported in the literature. Herein, we present 18 F-FDG PET/CT and 68 Ga-DOTATATE PET/CT findings of a unique case of primary extranodal MCL with gastric and adrenal involvement.

Correlation of low numbers of intratumoral FOXP3+ cells with worse progression-free survival in angioimmunoblastic T cell lymphoma

AimsAngioimmunoblastic T cell lymphoma (AITL) is a T cell lymphoma with aberrant immune activity. It is characterised by inflammatory and immune reactions. However, the impact of regulatory T (Treg) cells...

ALK-positive Anaplastic Large Cell Lymphoma Involving the Spinal Cord

Abstract Introduction/Objective ALK-positive anaplastic large cell lymphoma (ALCL) is a mature T-cell lymphoma that accounts for approximately 3% of non-Hodgkin lymphoma cases in the adult population. Lymph node involvement is most commonly seen, followed by extra-nodal involvement of the skin, soft tissue, and bone. Central nervous system involvement is rare at the time of diagnosis. This case study features a patient with systemic ALK-positive ALCL involving multiple nodal and extra-nodal sites, most notably the spinal cord. Methods/Case Report A 22-year-old female presented to the hospital with worsening back pain accompanied by neurological symptoms. MRI scans of her spine revealed extensive patchy abnormal marrow signal with enhancement of the thoracolumbar vertebrae (T1-L2) and adjacent extension into the spinal canal (T3-T7, T10-L1, L3-S1) and paraspinal soft tissues. A laminectomy of the T5-T7 and T11-T12 levels was performed for surgical decompression, and epidural and paraspinal resection specimens were submitted. These specimens, along with an excisional axillary lymph node biopsy, demonstrated ALK-positive ALCL after comprehensive immunohistochemical studies. In-situ hybridization for EBV was negative. CHOP therapy was initiated, but treatment was complicated by Candidemia and disseminated intravascular coagulation. The patient rapidly declined and passed away less than a month after hospital admission. At autopsy, enlargement of lymph nodes, the left ovary (17.8 g), and spleen (383.6 g) were noted on gross examination. A solid, white-tan lesion (2.1 x 0.9 x 0.8 cm) was found in the left ovary. Necrosis and infiltrates of large, pleomorphic lymphoid cells with irregular nuclear contours and prominent nucleoli were seen on microscopic examination of the spinal cord and leptomeninges, vertebral bone marrow, mediastinal lymph nodes, spleen, and left ovary. Consistent with previously submitted surgical specimens, the viable neoplastic cells stained strongly positive for ALK-1, CD4, and CD30. Results (if a Case Study enter NA) NA Conclusion This case study highlights an atypical presentation of ALCL with a fatal outcome. Given the availability of effective treatment options, prompt diagnosis is crucial to improving survival outcomes. Additional research is necessary to elucidate the clinical and pathological features of ALCL. The differential for back pain is broad, but including this entity for patients with similarly insidious clinical presentations can prevent a delay in diagnosis that allows for rapid progression of disease.

Low magnesium levels and prognosis in newly diagnosed diffuse large B-cell lymphoma.

Magnesium (Mg) is an essential element involved in cellular metabolism. We demonstrated that in patients with diffuse large B-cell lymphoma (DLBCL) undergoing autologous stem cell transplant (SCT), those with a serum Mg < 2.0 mg/dL at the time of transplant had worse outcomes. In this study, we aimed to learn the prognostic value of low serum Mg in patients with untreated DLBCL. We analyzed serum from 408 patients and tested 2 Mg cutpoints-low (<1.7 mg/dL) and low normal (<2.0 mg/dL), a range we found associated with lower survival in the SCT group. We found 3% of patients with low levels and 23% with low normal levels. Low normal serum Mg levels were associated with a higher stage at diagnosis, more extranodal involvement, higher international prognostic index score, lower overall survival (OS), and event-free survival. These data warrant testing Mg replacement to a target of >2.0 mg/dL to learn if survival can be improved.

Effect of Endothelial Activation and Stress Index(EASIX) on Prognosis of Peripheral T-Cell Lymphoma Patient

To investigate the effect of endothelial activation and stress index (EASIX) on the prognosis of patients with angioimmunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), and to compare the clinical characteristics of patients in the low EASIX and high EASIX groups. The clinical data of 59 newly diagnosed AITL and PTCL-NOS patients admitted to the Affiliated Hospital of Xuzhou Medical University from January 2010 to September 2021 were retrospectively analyzed. The optimal cut-off value of EASIX was determined by receiver operating characteristic (ROC) curve; The chi-square test was used to analyze the correlation between EASIX and clinical features of patients with AITL and PTCL-NOS; The Kaplan-Meier survival curve was used to analyze the overall survival (OS) and progression-free survival (PFS) of the patients; Univariate and multivariate analyses were performed by using Cox proportional hazards model. The optimal cut-off value of EASIX was 0.95, based on which the patients were divided into a low EASIX (<0.95) group and a high EASIX (≥0.95) group. Compared with the low EASIX group, the high EASIX group had a higher proportion of patients with advanced Ann Arbor stage, higher risk according to IPI, elevated LDH, hypoproteinemia, anemia, B symptoms,extranodal involvement, and bone marrow involvement. Survival analysis showed that the OS and PFS of patients in the high EASIX group were significantly shorter than those in the lower EASIX group(P <0.001). The multivariate analysis showed that EASIX was an independent risk factor for OS ［HR=7.217 (95%CI : 1.959-26.587), P =0.003］ and PFS ［HR=2.718(95%CI : 1.032-7.161), P =0.043］ of PTCL patients. High EASIX in newly diagnosed patients with AITL and PTCL-NOS suggests a poor prognosis, and high EASIX is a risk factor affecting prognosis of the patients.

A case report of orbital Castleman’s disease

Castleman's disease is a group of atypical lymphoproliferative disorders. Extranodal involvement is rare. We report a case of a 39-year male who had left painless proptosis for three months. Examination revealed a firm mass in the left inferior orbit, displacing the globe superiorly. MRI showed an irregular mass causing enlargement of the left recti muscles. Systemic workup was unremarkable. The mass was excised via inferior orbitotomy. Histopathology showed hyaline vascular variant of Castleman's disease. Postoperatively, proptosis resolved and no recurrence was noted. This highlights the importance of including CD while evaluating irregular orbital lesions for early detection and management.

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.

Correlations between Maximum Standardized Uptake Value Measured via 18 F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Clinical Variables and Biochemical Indicators in Adult Lymphoma

Abstract Background Lymphoma comprises a histological heterogeneous group of cancers derived from the cells of the immune system manifested as enlargement and proliferation of lymph nodes or secondary lymphoid tissues. Earlier, the imaging evaluation and follow-up of non-Hodgkin’s lymphoma patients were based solely on findings at contrast-enhanced CT. Fortunately, 18F- fluorodeoxyglucose (18F -FDG) positron emission tomography (PET)/computed tomography is gaining popularity for evaluating patients with non-Hodgkin lymphomas (NHLs) lymphoma and has proven to be more sensitive and specific than contrast-enhanced CT for evaluating nodal and extra-nodal lymphomatous involvement. Main body of Abstract 18 F-FDG PET/CT and the maximum standardized uptake value (SUV max) is currently used for the diagnosis and initial staging of NHLs. The potential usefulness of (SUV max) in NHL cases and the significance of correlations between SUV max and various indicators arc currently unknown. The aim of the current study was to investigate whether the (SUV max) measured by 18 F -FDG PET/CT could discriminate between aggressive and indolent (NHLs) including different pathological subtypes and analyze the correlations between the SUV max and other important parameters. A study was conducted among newly diagnosed 60 adult NHL patients confirmed by biopsy, pretreatment 18F-FDG PET/CT scans, and a complete medical record were retrospectively enrolled in the study including evaluation of various clinical variables; clinical stage, pathological subtype, international Prognostic Index (IPI) score as well as lactate dehydrogenase (LDH) value and then correlated with SUV max of the biopsy site on PET/CT. The area under the ROC curve for examining the accuracy of SUV max regarding distinguishing between aggressive and indolent NHLs was 0.85 and a cutoff value of &amp;gt; 7.7 yielded a sensitivity of 90 % and specificity of 73 %. The SUV max mean ± SD of aggressive NHL was significantly higher than that of the indolent NHL (P &amp;lt; 0.001). The SUV max of the biopsy site was strongly positively correlated with IPI score and clinical staging (P = 0.001), but it was not significantly correlated with LDH (P &amp;gt; 0.05). Short Conclusion 18F-FDG PET/CT may yield reliable measurements of tumor proliferation, and an SUV max &amp;gt; 7.7 suggesting that SUV max was a useful predictor of diagnosis and may distinguish between aggressive and indolent newly diagnosed NHLs in adults, SUV max correlates with IPI score and clinical staging. SUV max doesn’t correlate to LDH.

Identification, risk factors, and clinical course of CNS relapse in DLBCL patients across 19 prospective phase 2 and 3 trials-a LYSA and GLA/ DSHNHL collaboration.

Progression or relapse in the central nervous system (CNS) remains a rare but mostly fatal event for patients with diffuse large B-cell lymphoma (DLBCL). In a retrospective analysis of 5189 patients treated within 19 prospective German and French phase 2/3 trials, we identified 159 patients experiencing a CNS event (relapse: 62%, progression: 38%). Intracerebral, meningeal, intraspinal, or combined involvement was reported in 44%, 31%, 3%, and 22% of patients, respectively. 62 of 155 evaluable patients (40%) showed concurrent systemic progression/ relapse. 82% of all CNS events occurred within two years after study inclusion or randomization. 87% of patients showed extranodal involvement outside the CNS. Patients generally had poor outcomes with a median overall survival (OS) of 3.4 months (95% CI 2.9-4.2) and a 2-year OS of 15% (10-22%). Outcomes did not differ depending on the site or time point of CNS events. Patients with isolated CNS events demonstrated significantly better OS (p = 0.023). Twenty-five patients were consolidated with autologous or allogeneic stem cell transplantation and achieved a 3-year OS of 36% (20-66%). This large study including more than 5000 DLBCL patients highlights the unmet medical need to improve the outcome of DLBCL patients suffering from CNS relapse.