Lymphoma Research Articles

Exploring the Role of FDG PET CT Scan in Detecting High Grade Diffuse Large B-Cell Lymphoma

Introduction. Diffuse B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin’s lymphoma. Currently, the standard method for evaluating patients at the initial stages of cancer diagnosis in Mashhad oncology centers involves computed tomography scans (CT scans), histopathological evaluation of tissue, bone marrow sampling, and cytogenetic studies, all of which are time-consuming and costly. It is worth mentioning that at present, the most recommended approach for determining lymphoma staging is the FDG-PET/CT scan, which combines labeled glucose with CT scan and offers a more accurate alternative. The objective of this study is to explore the potential of FDG-PET/CT scan as a tool for detecting high-grade lymphoma.Methods. In this study, patients with different types of DLBCL who underwent FDG-PET Scan for staging at Razavi Hospital, Mashhad, Iran between 2017 and 2021 were examined. The necessary clinical and paraclinical information, including the stage of the disease, the involved site at the time of diagnosis, the result of immunohistochemical examination, and the response to treatment were collected. FDG-PET Scan information including the extent of involvement and metabolic activity of the tumor before the start of treatment, pathological characteristics of the tumor, clinical behavior, and response to treatment in the form of response rate (RR), disease-free survival (DFS) and overall survival (OS) of the patients. Was also investigated. Aggressive histology in the present study was classified based on morphological characteristics and immunohistochemical staining, prognostic indicators, clinical behavior and response to treatment. Data were analyzed using SPSS software at a significance level of p<0.05.Results. Comparing the two groups of patients with high grade histology (n=12) and NOS (n=14), the results showed that SUV max values in patients with aggressive lymphoma were 27.5 ± 15.6 (median 25.6) and in patients with NOS lymphoma was 15.4 ± 9.8 (median 14.4) (p=0.01). The overall survival of patients in the aggressive group was 10 months and in the non-aggressive group was 24 months (p=0.002). Also, the cut — off -point of 21.1 for SUV max has a sensitivity of 66 % and a specificity of 72 % in differentiating aggressive from non-aggressive types.Conclusion. The results revealed that FDG PET CT Scan can provide valuable insights into differentiating lymphomas with a more aggressive type from their usual types, as those with heightened metabolic activity (SUVmax) are often indicative of aggressive behaviors.

Casual detection of an abdominal aortic aneurysm thanks to a trainee-made ultrasound with two subsequent "domino" diagnoses: case report and insights on the role of ultrasound courses.

Early detection of abdominal aortic aneurysm (AAA) is crucial as untreated lesions can be life-threatening. We describe the case of a fortuitous ultrasound (US) detection of AAA by a trainee which allowed two subsequent "domino" diagnoses. A 69 y.o. male underwent US to evaluate prostate and bladder. He was a heavy smoker affected by hypertension and dyslipidemia. An endocrinology resident and ultrasound trainee started the exam accidentally from the epigastrium and found a fusiform aneurysmal lesion. Subsequent computed tomography (CT) scan confirmed the diagnosis of AAA and incidentally revealed a colon adenocarcinoma and a diffuse non-Hodgkin lymphoma. This case report underlines the importance of extending the abdominal US examination to the aorta in patients over 60years of age (especially in those people with multiple risk factors for AAA) and emphasizes the critical role of organizing hands-on-patient US courses with a multidisciplinary approach to optimize the training of future sonographers.

A case of malignant lymphoma of the extrahepatic bile duct diagnosed by detailed imaging examination and endoscopic ultrasound-guided fine needle aspiration.

A 70-year-old woman presented to our hospital with abdominal pain. Imaging examinations showed diffuse and extensive wall thickening at the perihilar bile duct; however, the degree of stricture was mild, and the mucosal epithelium was smooth. A transpapillary biopsy was performed considering cholangiocarcinoma and IgG4 sclerosing cholangitis as differential diagnoses; however, no pathologic diagnosis was obtained. Peroral cholangioscopy revealed a regular epithelium at the stricture, and endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) of the enlarged lymph node confirmed the diagnosis of diffuse large B-cell lymphoma. Multiagent chemotherapy was administered, which led to complete remission. Because primary bile duct malignant lymphomas are rare and specific, clinical, and imaging findings are lacking, and many of those reported so far have been diagnosed by postoperative pathology. As chemotherapy is the first-line treatment for malignant lymphoma, obtaining an accurate diagnosis is crucial. Our findings support that smooth and mild biliary strictures with mainly submucosal wall thickening may be characteristic imaging findings of primary bile duct malignant lymphoma, and that peroral cholangioscopy and EUS-FNA may be helpful for an accurate diagnosis.

Usefulness of the 3-hydroxykynurenine/kynurenic acid ratio as a diagnostic biomarker for diffuse larger B-cell lymphoma.

Reports have shown that the kynurenine pathway, one of the pathways by which tryptophan is metabolized, is activated in patients with diffuse large B-cell lymphoma (DLBCL). Activation of the kynurenine pathway triggers the production of various metabolites, such as kynurenine (Kyn), 3-hydroxykynurenine (3-HK), 3-hydroxyanthranilic acid (3-HAA), kynurenic acid (KA), and anthranilic acid (AA), which contribute to immune tolerance. The current study aimed to investigate the changes in metabolites of kynurenine pathway in DLBCL patients and evaluate their performance predicting DLBCL. Changes in metabolites of kynurenine pathway were examined using high-performance liquid chromatography in 35 DLBCL patients (age 61.2 ± 13.5years) and 44 healthy controls (age 58.5 ± 12.5years). DLBCL patients had significantly higher levels of 3-HK, AA, and 3-HAA but lower levels of tryptophan (Trp) and KA compared to healthy controls. Given that the ratio of each metabolite represents the change in the Kyn pathway, the 3-HK/KA ratio was examined. Notably, DLBCL patients had a significantly higher 3-HK/KA ratio compared to healthy controls. In DLBCL, the area under the receiver operative characteristic (ROC) curve for 3-HK/KA (0.999) was higher than that for lactate dehydrogenase (0.885) and comparable to that for soluble interleukin-2 receptor (sIL-2R) (0.997). Based on ROC curve analysis, the 3-HK/KA ratio was found to be useful biomarker for the diagnosis of DLBCL. Our results suggest that the 3-HK/KA ratio is a clinically useful biomarker of DLBCL. Moreover, its combination with existing markers, such as sIL-2R, can improve its effectiveness of diagnosing DLBCL.

Epstein-Barr Virus Latent Membrane Protein 1 Subverts IMPDH pathways to drive B-cell oncometabolism.

Altered metabolism is a hallmark of cancer, yet much remains to be learned about how EBV rewires host cell metabolism to support multiple malignancies. While the oncogene LMP1 is the only EBV-encoded gene that is sufficient to transform murine B-cells and rodent fibroblasts, knowledge has remained incomplete about how LMP1 alters host cell oncometabolism to aberrantly drive infected B-cell growth and survival. Likewise, it has remained unknown whether LMP1 expression creates metabolic vulnerabilities that can be targeted by small molecule approaches to trigger EBV-transformed B-cell programmed cell death. We therefore used metabolomic profiling to define how LMP1 signaling remodels the B-cell metabolome. We found that LMP1 upregulated purine nucleotide biosynthesis, likely to meet increased demand. Consequently, LMP1 expression sensitized Burkitt B-cells to growth arrest upon inosine monophosphate dehydrogenase blockade. Thus, while LMP1 itself may not be a therapeutic target, its signaling induces dependence on downstream druggable host cell nucleotide metabolism enzymes, suggesting rational therapeutic approaches.

Preliminary Efficacy of Orelabrutinib Targeted Therapy in Induction Therapy of Primary Central Nervous System Diffuse B-Cell Lymphoma

Preliminary Efficacy of Orelabrutinib Targeted Therapy in Induction Therapy of Primary Central Nervous System Diffuse B-Cell Lymphoma

Protein-truncating and rare missense variants in ATM and CHEK2 and associations with cancer in UK Biobank whole-exome sequence data

BackgroundDeleterious germline variants in ATM and CHEK2 have been associated with a moderately increased risk of breast cancer. Risks for other cancers remain unclear.MethodsCancer associations for coding variants in ATM...

High-grade diffuse large B cell non-Hodgkin lymphoma involving the mucosal hemorrhoid tissue: A case report

Non-Hodgkin's lymphomas are a diverse group of immune system cancers that can affect any organ in the body. Few reports describe the development of malignant lymphoma in internal or external hemorrhoids. We present the case of a 67-year-old female with a long-standing history of non-bleeding, prolapsing hemorrhoids, and recent significant weight loss. Following an excisional hemorrhoidectomy, pathological analysis revealed high-grade diffuse large B-cell non-Hodgkin lymphoma (DLBCL) involving the mucosal hemorrhoid tissue. The case underscores the importance of considering malignancy in atypical presentations of common conditions. Treatment for anal canal lymphomas remains uncertain, with medical management generally preferred over surgery. Accurate diagnosis requires histopathologic examination due to the overlap of clinical features with other pathologies.

Closing the gap between biology and classification in splenic B-cell lymphomas.

The mature splenic B-cell lymphomas are an enigmatic group of lymphoid neoplasms that have long caused significant difficulty for the practicing pathologist due to overlapping diagnostic features among entities and the decreasing availability of splenic tissue for assessment. While some entities have highly characteristic and specific clinicopathological features (e.g. hairy cell leukaemia), others are substantially more difficult to recognise (e.g. splenic diffuse red pulp lymphoma). At the same time, classification systems have been evolving, resulting in multiple changes to the boundaries among these entities and even the existence of some entities in their own right. Moreover, unbiased multi-omic interrogation (whole genome/transcriptome sequencing, methylome) of the splenic B-cell lymphomas over the past decade has given us significant insights into the underling biology of these neoplasms. We present a clinicopathological perspective on the historical, current and future state of the diagnosis and classification of splenic B-cell lymphomas integrating multi-omic data and highlighting areas of focus for the field in order to continue to strive to improve patient outcomes through accurate diagnosis.

Efficacy of thiotepa-conditioned autologous stem-cell transplantation as consolidation therapy for primary leptomeningeal malignant lymphoma: a case report and review of literature.

Primary leptomeningeal malignant lymphoma (PLML) is a rare subtype of primary central nervous system lymphoma (PCNSL). Treatment is often based on PCNSL, but currently there is no established treatment strategy due to its rarity. We report a case of a 46-year-old male diagnosed with PLML through cerebrospinal fluid cytology and flow cytometry, presenting with multiple cranial nerve palsies and L5 radiculopathy. The patient achieved complete remission (CR) with R-MPV (rituximab, methotrexate, procarbazine, and vincristine) combined with intrathecal chemotherapy (methotrexate, cytarabine, and prednisolone). This was followed by autologous stem-cell transplantation (ASCT) using a thiotepa-based conditioning regimen, resulting in sustained CR. A literature review on the use of ASCT for PLML revealed three reported cases, including ours, all achieving CR with ASCT and minimal adverse events. These findings suggest that ASCT can be a promising consolidation therapy for PLML. Further studies are needed to establish standardized treatment protocols for this rare condition.

Ciliary body myxoid epithelioid sarcoma in a cat: a case report

BackgroundThe majority of primary, intraocular tumors in cats originate from the uvea and include feline diffuse iris melanoma, lymphoma, and iridociliary epithelial adenoma or adenocarcinoma. In this case report, we describe for the first time the clinical, histological, and immunohistochemical findings of a rare myxoid intraocular neoplasm arising from the ciliary body in a cat.Case presentationA 14-year-old, female, spayed domestic shorthaired cat was presented for evaluation of discolouration of the right eye. Upon examination, a clear to light whitish-tan, bubble-shaped intraocular mass adherent to the inferior ciliary body and extending into the anterior chamber was noted. Within five weeks, the tumor was significantly larger and the eye had developed secondary glaucoma so was enucleated. Light microscopic examination of the globe revealed a multinodular, hypocellular neoplasm arising from the ciliary body composed of interwoven spindle cells embedded in abundant amounts of a lightly basophilic myxoid matrix. Neoplastic cells exhibited strong immunoreactivity for cytokeratin while also showing moderate to strong immunoreactivity to vimentin. A diagnosis was therefore made of an unusual intraocular myxoid epithelioid sarcoma arising from the ciliary body.ConclusionsAlthough apparently exceedingly rare, epithelioid myxosarcoma should be included as a differential diagnosis for intraocular tumors in cats and they represent a clinical, histologic, and immunohistochemical diagnostic challenge. Early surgical intervention should be considered to prevent local invasion and ascension to the brain.

P16-7 Case report: a diffuse large B-cell non-Hodgkin lymphoma mixed Follicullar lymphoma in elderly with complete response

P16-7 Case report: a diffuse large B-cell non-Hodgkin lymphoma mixed Follicullar lymphoma in elderly with complete response

Understanding splenic B-cell lymphoma/leukaemia with prominent nucleoli: Diagnosis, underpinnings for disease classification and future directions.

The 5th edition of the WHO classification of haematolymphoid tumours (WHO-HAEM5) introduced a new category, splenic B-cell lymphoma/leukaemia with prominent nucleoli (SBLPN). The diagnostic entity B-cell prolymphocytic leukaemia (B-PLL) has been discontinued and the category of hairy cell leukaemia variant (HCLv) has been conceptually reframed. B-PLL and HCLv diagnoses were uncommon. Overlap existed between B-PLL and other indolent lymphomas like chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL). HCLv lacked consistent cytomorphological, immunophenotypic and genetic features. To address these issues, the WHO-HAEM5 classification has introduced SBLPN to serve as a temporary holding ground for entities that do not neatly fit into the existing classification. Cases previously classified as CD5-negative B-PLL and HCLv fall under the SBLPN category. Some splenic marginal zone lymphoma and splenic diffuse red pulp small B-cell lymphoma cases with higher number of medium or large nucleolated B cells would also be classified as SBLPN under the WHO-HAEM5. This review explores the rationale for discontinuing B-PLL and HCLv diagnoses. It then examines the concept of SBLPN, offers practical guidance for diagnosis and discusses future directions in classifying splenic B-cell lymphomas.

Splenic cyst deroofing complicated with B lymphoma

BackgroundSplenic cysts are uncommon and very rarely malignant therefore their treatment isn’t standardized. In case of symptomatic cysts different surgical approaches have been suggested. Primary malignant lymphoma of the spleen comprises less than 1% of non-Hodgkin’s lymphomas. To our knowledge, only 203 cases of splenic large B-cell lymphoma (LBCL) have been reported to date and only 2 of them were fibrin-associated splenic cysts.Case presentation27-year-old model with a 19 × 13 cm splenic cyst without data of malignancy in the preliminary study and therefore treated with laparoscopic deroofing. After histological diagnosis of LBCL with a fibrin/EBV-associated splenic pseudocyst, the patient received 4 cycles of Rituximab and a laparoscopic splenectomy was performed due to resurgence of the pseudocyst. No evidence of malignancy has been found during follow up (EBV viral load every 3 months during the first year, PET-CT every 6 months during the first year and annual afterwards) performed after the splenectomy.Discussion and conclusionsThe value of tumor markers and radiology for diagnosis of splenic cysts is put into question.Only 60 cases of Fibrin-associated LBCL (FA-LBCL) have been described in the literature therefore there are no treatment guidelines for them even though surgery together with systemic treatment has been the prevalent route with good results in most cases.

Plea to radiologists: Please consider Mahvash disease when encountering an enlarged pancreas.

Radiologists play a key role in establishing an early and accurate diagnosis, especially for rare diseases. Mahvash disease (OMIM 619290) is an autosomal recessive hereditary disease caused by inactivating mutations of the glucagon receptor and its main clinical consequences are pancreatic neuroendocrine tumors and in some cases, porto-sinusoidal vascular disease and portal hypertension. Untreated Mahvash disease can be lethal. The diagnosis of Mahvash disease has almost always been delayed in the past due to radiologists' unawareness of or unfamiliarity with the unique imaging features of Mahvash disease which are moderately to enormously enlarge pancreas with preserved pancreas contour and parenchyma without vascular involvement or lymphadenopathy. These features help differentiate Mahvash disease from other etiologies of diffusely enlarged pancreas such as diffuse pancreatic ductal carcinoma, diffuse pancreatic lymphoma, and autoimmune pancreatitis. Invoking Mahvash disease in the differential diagnosis of an enlarged pancreas has recently been shown to facilitate early diagnosis. To prevent missing the diagnosis of this significant disease, I sincerely ask radiologists to consider Mahvash disease in their differential diagnoses of diffusely enlarged pancreas.

A constellation of cutaneous reactions to chimeric antigen receptor T-cell therapy.

This case report describes a cascading purpuric eruption progressing to bullae, ulceration and hypergranulation in a 76-year-old patient with diffuse B-cell lymphoma, following chimeric antigen receptor T-cell therapy. It is crucial to document and report these cutaneous reactions to raise awareness, improve management and monitor patient outcomes.

Clinical Characteristics and Treatment Outcomes of Pediatric Patients with Non-Hodgkin Lymphoma: a Single-Center Experience From Southern Turkey

Purpose: Non‑Hodgkin lymphoma (NHL) accounts for 8‑10% of all childhood cancers. In this study, we aimed to describe the epidemiological and clinical characteristics and treatment outcomes of pediatric NHL patients treated at a tertiary center. Methods: The oncologic records of the patients diagnosed and followed up as NHL between 2013 and 2023 were reviewed retrospectively. Results: A total of 36 patients were enrolled in this study. The most common pathologic subtype was lymphoblastic lymphoma (LL) (n=21, 58.3%) followed by Burkitt lymphoma (BL) (n=10, 27.8%), diffuse large B-cell non-Hodgkin lymphoma (DLBCL) (11.1%, n=4), and anaplastic large cell lymphoma (ALCL) (2.8%, n=1). Overall survival (OS) and event-free survival (EFS) were significantly longer in patients without bone marrow (BM) involvement (p=0.001 and p=0.02, respectively). EFS was significantly longer in patients without central nervous system (CNS) involvement (p=0.038). OS and EFS did not differ significantly according to NHL subtypes (p>0.05). There was no significant difference in OS according to age groups (p=0.7). Conclusion: The OS with NHL has significantly improved. With the development of effective treatment regimens based on various pathologic subtypes, the result of pediatric NHL has significantly improved in recent years. The survival rates have reached >90%.

Enhancer-driven gene regulatory networks inference from single-cell RNA-seq and ATAC-seq data.

Deciphering the intricate relationships between transcription factors (TFs), enhancers, and genes through the inference of enhancer-driven gene regulatory networks (eGRNs) is crucial in understanding gene regulatory programs in a complex biological system. This study introduces STREAM, a novel method that leverages a Steiner forest problem model, a hybrid biclustering pipeline, and submodular optimization to infer eGRNs from jointly profiled single-cell transcriptome and chromatin accessibility data. Compared to existing methods, STREAM demonstrates enhanced performance in terms of TF recovery, TF-enhancer linkage prediction, and enhancer-gene relation discovery. Application of STREAM to an Alzheimer's disease dataset and a diffuse small lymphocytic lymphoma dataset reveals its ability to identify TF-enhancer-gene relations associated with pseudotime, as well as key TF-enhancer-gene relations and TF cooperation underlying tumor cells.

Intraocular manifestation of a diffuse large B-cell non-Hodgkin lymphoma of the pancreas.

Intraocular manifestation of a diffuse large B-cell non-Hodgkin lymphoma of the pancreas.

De novo primary malignant lymphoma of the dura following recurrent episodes of subdural abscess presenting as chronic subdural hematoma: illustrative case.

Chronic inflammation of the thorax, as in tuberculosis-related pyothorax, can cause secondary malignant lymphomas. However, primary malignant lymphoma of the central nervous system, specifically of the dura mater, developing after intracranial infection or inflammation has rarely been reported. Herein, the authors describe a case of primary dural lymphoma that developed secondary to subdural empyema, with an initial presentation mimicking a chronic subdural hematoma. A 51-year-old man had undergone single burr hole drainage for subdural empyema 2 years prior. The patient subsequently underwent multiple craniotomy and drainage procedures, with successful remission of the subdural empyema. He was subsequently referred to the authors' hospital approximately a year after his initial treatment because of a recollection of subdural fluid, which was suspected to be recurrent empyema. After another single burr hole drainage, which revealed only a subdural hematoma, a histopathological diagnosis of B-cell lymphoma of the dural/subdural membrane was made. Subsequent radiation therapy was completed, with good local control and no recurrence of the subdural hematoma confirmed at 2 months posttreatment. Intracranial lymphoma triggered by chronic inflammation is rare but should be considered a differential diagnosis in subdural hematomas for which the background pathology is unclear. https://thejns.org/doi/10.3171/CASE24153.