Explore the vast range of titles available.

Abstract Objectives To assess the prevalence of Hashimoto thyroiditis (HT) in primary thyroid lymphoma (PTL) and whether it differs between mucosa-associated lymphoid tissue (MALT) lymphoma and diffuse large B-cell lymphoma (DLBCL). Methods Electronic databases were searched for studies assessing HT prevalence in PTL, based on antithyroid antibodies, clinical history, or pathology. Pooled prevalence of HT and its association with histotype (MALT or DLBCL) were calculated. Results Thirty-eight studies with 1,346 PTLs were included. Pooled prevalence results were 78.9% (any HT evidence), 65.3% (antithyroid antibodies), 41.7% (clinical history), and 64% (pathology). HT prevalence was significantly higher in MALT lymphoma than in DLBCL (P = .007) and in mixed DLBCL/MALT than in pure DLBCL (P = .002). Conclusions Overall, 78.9% of patients with PTL have any HT evidence, but only half of these had been clinically followed. The difference in HT prevalence suggests that a subset of DLBCL may not derive from MALT lymphoma.

Immunological and serological tests revealed a C-reactive protein level of 1.35 mg/dL, a soluble form of the interleukin-2 receptor (sIL-2R) level of 1070 U/mL, negative hepatitis C virus antibodies, negative antinuclear antibodies, negative anti-aminoacyl tRNA synthetase (ARS) antibodies, and positive transcription intermediary factor 1-gamma (anti-TIF-1γ) antibodies (91; index < 32). The prevalence of malignancy in anti-TIF-1γ antibody-positive dermatomyositis is 40.7% (95% CI: 36–45%), with an estimated sensitivity of 52% (95% CI: 47–57%), specificity of 92% (95% CI: 90–93%), and positive likelihood ratio of 4.2.5 The prevalence of hematologic malignancies in anti-TIF-1γ antibody-positive dermatomyositis is 1% (95% CI: 0 − 8%), which is low in comparison to the prevalence of solid tumors (56%, 95% CI: 46–66%).5 Characteristic clinical findings of this condition include extensive erythema on the scalp, face, neck (V-neck sign), and back, as well as dysphagia. Extensive erythema and dysphagia are associated with malignancies and the presence of anti-TIF-1γ antibodies.6,7 In patients with dermatomyositis, dysphagia may be associated with or precede dysphonia, as seen in this case, making them useful clinical signs.8 Additionally, cases of dermatomyositis with vesicular rash are generally associated with malignancies, raising the possibility of a link between vesicle formation and anti-TIF-1γ antibodies.9 Previous case reports suggest that malignancy-associated dermatomyositis generally improves following the treatment of the malignancy, even in anti-TIF-1γ antibody-positive cases.10 Therefore, in anti-TIF-1γ antibody-positive dermatomyositis, concurrent diagnosis and treatment of malignancies and dermatomyositis may lead to an improved prognosis. Recently, IVIG has been shown to have a favorable safety profile for the treatment of adult patients with dermatomyositis and is becoming an established treatment for dermatomyositis.11 In addition, administering IVIG in the early stages might prevent the emergence of muscle weakness.12 18F-FDG PET-CT has been reported to be useful as a screening test for malignancies in patients with anti-TIF1γ antibody-positive dermatomyositis who present at age 40 years or older and have clinical signs suggesting a high risk of malignancy.13 In this case, the persistence of high disease activity despite immunosuppressive therapy and the presence of moderate to severe dysphagia were clues to the presence of a malignancy.14 Limiting PET-CT to cases with a high risk of malignancy has the potential to reduce the risk of false-positive diagnoses, and identify malignancy in a single test, thus

The number of people with HIV (PWH) in Africa is rising due to population growth and antiretroviral therapy (ART) availability, with diffuse large B-cell lymphoma (DLBCL) a major cause of mortality. HIV and ART alter DLBCL tumor biology, but few studies of DLBCL include PWH or African patients, limiting translation of emerging treatment strategies. Here, we performed whole exome sequencing of 48 tumors (40 HIV-positive [HIV+]) with paired germline of DLBCL patients from Malawi and South Africa. HIV + DLBCL tumors had distinct mutations depending on ART exposure, and there were several recurrent deleterious variants, with ANKRD11 mutations being prognostic. One tumor from each cohort had high tumor mutational burden and microsatellite-instability with PMS2 and ARID1A mutation. These findings suggest shared genomic characteristics among HIV + DLBCL in Africa, offering opportunities for tailored biomarkers and therapeutic targets for this underserved population.

Background Splenic 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 presentation 27-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 conclusions The 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.

Axicabtagene ciloleucel (Axi-cel) is a CD-19 Chimeric Antigen Receptor T cell therapy approved for the treatment of relapsed/refractory diffuse large B cell lymphoma. We treated ten patients with DLBCL post-FDA approval in an inner-city tertiary center in the Bronx. Eight patients (80%) had received ≥ 3 lines of therapy, six patients had received prior radiation, and seven had recurrent disease after prior autologous hematopoietic stem cell transplant (AHCT). Our cohort included one patient with HIV, two patients with hepatitis B, and two patients with CNS involvement of lymphoma. Axi-cel treatment led to significant responses with 8/10 patients achieving a complete remission at 3 months, including both patients with prior CNS involvement. The treatment was generally well tolerated with 20% of patients experiencing grade ≥ 2 CRS. One patient each with HIV and hepatitis B responded without significant toxicities. In conclusion, Axi-cel led to significant efficacy with manageable toxicity in DLBCL in a real-world setting.

Neurolymphomatosis (NL) is a rare manifestation of lymphoma resulting from the infiltration of malignant lymphoma cells into the peripheral nervous system. When evaluating peripheral neuropathy, clinical suspicion of NL is highly necessary because its clinical symptoms vary and pathological diagnosis is often difficult. Imaging modalities carry a high sensitivity to diagnose NL. 18 F-flurodeoxyglucose positron emission tomography-computed tomography (18 F-FDG PET/CT), with a diagnostic sensitivity of over 90%, as well as Magnetic resonance imaging (MRI) neurography, has become the leading diagnostic modalities for NL diagnosis, staging, and treatment response assessment. At present, the prognosis of NL is usually poor, and its treatment is also challenging, with few patients benefiting from the currently available treatment measures. The present report discusses two cases of diffuse large B-cell lymphoma-associated NL. They presented with painful polyneuropathy/polyradiculopathy, and imaging examinations such as MRI and 18 F-FDG PET/CT indicated related neuropathy. Both cases were treated with PR2-based immunotherapy (anti-PD-1 tirelizumab 200 mg d1, rituximab 375 mg/m 2 d0, and lenalidomide 25 mg d1–14). And clinical efficacy was observed, with improved symptoms. Imaging studies showed a significant reduction in lesions of the cervical plexus nerve roots, lumbosacral nerve, sciatic nerve, and brachial plexus. In this study, the two cases of NL with typical clinical and imaging manifestations discussed in the present report exhibited good clinical efficacy after receiving PR2-based immunotherapy. These results provide insights into the development of a novel, potentially effective treatment option for patients with NL.

The occurrence of diffuse large B-cell lymphoma (DLBCL) in the course of Sjogren’s syndrome (SS) is considered to be equally related either to the development of DLBCL de novo or to the transformation from marginal zone lymphoma (MZL). However, the question of possible clonal relationship between MZL and DLBCL in the group of SS patients remains open. Here we present the data concerning 194 patients with lymphoma complicated SS followed up at Nasonova Research Institute of Rheumatology during the last 22 years. Molecular analysis of tumor cells was performed for 6 SS patients who had developed both MZL and DLBCL. To assess clonal relationship between each of the tumor pairs immunoglobulin heavy chain (IGH) gene rearrangements were identified according BIOMED-2 protocol by means of multiplex polymerase chain reaction followed by GeneScan fragment analysis. Despite different localization MZL and DLBCL were clonally related in five tumor pairs. The median time to transformation was 11 months (range 0–78 months). MZL and DLBCL were clonally related in most cases from our cohort of SS patients. No statistically significant difference in survival between patients with DLBCL transformed from MZL and patients with de novo DLBCL was found in the cohort of SS patients investigated.

Objective: To investigate the characteristics of central nervous system (CNS) involvement in children with non-Hodgkin’s lymphoma (NHL) and the value of flow cytometry (FC) in the diagnosis of CNS disease in pediatric NHL. Methods: The data of 56 newly diagnosed pediatric NHL patients with CNS involvement (CNS+/mass, CNS+/palsy, CNS+/CSF) were analyzed. The proportions and formats of CNS disease in different pathological types were compared. In addition, FC and conventional cytology (CC) of cerebrospinal fluid (CSF) were carried out in 383 newly diagnosed NHL cases. Results: A total of 383 children with NHL were enrolled. Among these patients, 56 (14.6%) were diagnosed with positive CNS involvement (CNS+), 33 had bulky disease (tumor diameter >10 cm), 32 had bone marrow invasion, 32 had lactate dehydrogenase levels >1000 U/L, and 25 had invasion of more than 4 organs at the time of diagnosis. There were 14 patients with T lymphoblastic lymphoma (T-LBL), 9 with B lymphoblastic lymphoma (B-LBL), 26 with Burkitt’s lymphoma (BL), and 2 with Epstein-Barr virus-positive diffuse large B cell lymphoma (EBV + DLBCL). Among the 56 CNS+ patients, 35 were CSF-positive (CSF+); there were 2 patients who were CSF+ via CC detection and 35 who were CSF+ via FC detection. The difference between CC and FC was statistically significant (P < 0.01). In the T-LBL group, 14 patients were CNS+/CSF, and in the B-LBL group, 8 were CNS+/mass. In the BL group, 22 patients were CNS+/mass and 15 were CNS+/CSF. In the anaplastic large-cell lymphoma group, 5 patients were CNS+/mass. Nine of the 56 CNS+ patients had events. The 2-year overall survival rate was 87% ± 0.046%, and the 2-year event-free survival rate was 76.2% ± 0.07%. Conclusion: CNS+ diagnoses were more common in pediatric NHL patients with bulky disease and/or bone marrow involvement and/or involvement of more than 4 organs at the time of diagnosis, and they were also common in the EBV + DLBCL and BL groups. FC of CSF showed important clinical significance in the diagnosis of CNS disease in pediatric NHL patients, and it can be used to significantly improve the CNS+ detection rate.

Hemophagocytic lymphohistiocytosis (HLH), as a life-threatening hyperinflammatory syndrome, rarely presents as a harbinger of aggressive large B cell lymphoma (LBCL), with a rapidly progressive clinical course and poor prognosis. A total of 30 patients diagnosed with aggressive LBCL concurrent with HLH were retrospectively reviewed in this study. Median age was 60 years (range, 24 to 85 years). Thirteen (43.3%) patients treated with ruxolitinib combined with corticosteroid (Ru-D) regimen achieved the highest overall response rate (ORR) of 84.6%, which was significantly higher than that of 40.0% in the etoposide and corticosteroid group and 33.3% in the corticosteroid group ( P  = 0.019). The median overall survival (OS) was 16.2 months, with corresponding 1-year and 2-year OS rates of 63.3% and 38.4%, respectively. The 8-week mortality rate was 26.7%. Patients responded to anti-HLH therapy within 2 weeks had significantly better OS than non-responsive group ( P  = 0.009). Low-intensity chemotherapy without anthracycline as the first-line of anti-lymphoma therapy followed by RCHOP did not compromise survival, and the median OS was 13 months and 19.1 months, respectively ( P  = 0.457). Ferritin levels ≥ 3606 ng/mL and uncontrolled HLH within 2 weeks were the independent risk factors associated with inferior OS. Our findings highlight the high early mortality and short survival of these patients and underscore the urgent need for developing more effective treatment strategies to improve prognosis.