2,678 results match your criteria: "Angioimmunoblastic Lymphoma"

Angioimmunoblastic T-cell lymphoma (AITL) is a kind of aggressive T-cell lymphoma with significant enrichment of non-malignant tumor microenvironment (TME) cells. However, the complexity of TME in AITL progression is poorly understood. We performed single-cell RNA-Seq (scRNA-seq) and imaging mass cytometry (IMC) analysis to compare the cellular composition and spatial architecture between relapsed/refractory AITL (RR-AITL) and newly diagnosed AITL (ND-AITL).

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Although rearrangement of the MYC oncogene (MYC-R) is frequently observed in aggressive B-cell lymphomas, it is extremely rare in T-cell malignancies. A 64-year-old man who had been under observation for several years because of asymptomatic pulmonary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALToma) was admitted to our hospital because of poor general condition and hypotension. Blood tests revealed thrombocytopenia and elevated serum lactate dehydrogenase levels, whereas computed tomography revealed systemic lymphadenopathy and splenomegaly.

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Article Synopsis
  • Adult T-cell leukemia/lymphoma (ATLL) is a type of cancer linked to HTLV-1 virus infection, with the WHO's new 5th Edition classification outlining essential and desirable diagnostic criteria for its identification.
  • A new diagnostic method combining HBZ-ISH and tax-PCR enhances detection of HTLV-1 in cases where only preserved tissue samples are available, alongside traditional Southern blot techniques.
  • The review addresses the morphological and phenotypic characteristics of ATLL, noting the prevalence of certain phenotypes, genetic abnormalities, and emphasizing the need for further research and classification in understanding the disease's complex nature.
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Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive subtype of peripheral T-cell lymphoma (PTCL) characterized by its T-follicular helper (TFH) phenotype. Relapsed and refractory disease is common in AITL and often associated with a poor prognosis. The presence of epigenetic abnormalities, immune dysregulation, hyperinflammation and active angiogenesis in AITL offers potential targets for histone deacetylase (HDAC) inhibitors and immunomodulatory drugs (IMiDs).

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The role of stem cell transplant (auto and allo) in PTCL and CTCL.

Hematology Am Soc Hematol Educ Program

December 2024

Department of Medicine A, University Hospital Muenster, Muenster, Germany.

In contrast to B-cell lymphoma, the advent of modern targeting drugs and immunotherapeutics has not led to major breakthroughs in the treatment of peripheral T-cell lymphoma (PTCL) to date. Therefore, both autologous and allogeneic hematopoietic cell transplantation (HCT) continue to play a central role in the management of PTCL. Focusing on the most common entities (PTCL not otherwise specified, angioimmunoblastic T-cell lymphoma, and ALK-negative anaplastic large cell lymphoma), we summarize evidence, indications, and points to consider for transplant strategies in PTCL by treatment line.

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Angioimmunoblastic T-cell lymphoma: Current Diagnostic Insights and Advances.

Hum Pathol

November 2024

Department of Pathology and Laboratory Medicine, Yale-New Haven Hospital, New Haven, CT, 310 Cedar Street, Ste BML 116C, New Haven, CT, 06510, USA. Electronic address:

Angioimmunoblastic T-cell lymphoma (AITL), or nodal T-follicular helper cell lymphoma, angioimmunoblastic type, is a rare and aggressive type of T-cell lymphoma characterized by a spectrum of clinical and histopathological features that can present diagnostic challenges. Derived from T-follicular helper cells, the genesis of AITL is thought to be a multistep process involving mutations in epigenetic regulatory genes such as TET2 and DNMT3A, followed by driver mutations in RHOA and IDH2 which promote clonal expansion as well as a characteristic inflammatory milieu. This review aims to provide a comprehensive overview of AITL, including its clinical presentation, epidemiology, pathogenesis, histomorphology and treatment options.

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Article Synopsis
  • Peripheral T-cell lymphoma (PTCL) includes various aggressive subtypes, with the PTCL-NOS and angioimmunoblastic types showing particularly poor responses to standard treatment.
  • Two unique cases illustrate how PTCL can present atypically, with one patient initially misdiagnosed with hip pain and another mistaken for a respiratory infection.
  • Both cases highlight the importance of recognizing constitutional symptoms and conducting thorough imaging to ensure timely diagnosis and treatment, ultimately leading to complete remission after chemotherapy.
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Background: Patients with relapsed or refractory (R/R) peripheral T-cell lymphoma (PTCL) generally have poor prognoses and limited treatment options.

Materials & Methods: This study evaluated the efficacy of a novel CD30/CD16A bispecific innate cell engager, acimtamig (AFM13), in patients with R/R PTCL. Patients included those with CD30 expression in ≥1% of tumor cells and who were R/R following ≥1 prior line of systemic therapy.

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The nonrandomized phase II study by Ding et al. explored the combination of azacitidine and chidamide with or without GemOx chemotherapy in relapsed/refractory peripheral T cell lymphoma and demonstrated that the dual epigenetic therapy is safe and efficacious, particularly in the angioimmunoblastic T cell lymphoma subset. Further investigation into adding chemotherapy is warranted, building on the promising results seen in this trial.

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  • The study aimed to evaluate how endothelial activation and stress index (EASIX) affect the prognosis of patients with angioimmunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS).
  • Researchers analyzed clinical data from 59 recently diagnosed patients to determine an optimal EASIX cut-off value and assess survival outcomes.
  • Findings revealed that a higher EASIX (≥0.95) correlates with more severe clinical features and significantly shorter overall survival and progression-free survival, indicating it as an independent risk factor for worsened prognosis.
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  • Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of non-Hodgkin lymphoma that can show various clinical symptoms, and this report discusses a specific case where it was accompanied by pulmonary arterial hypertension (PAH).
  • An 84-year-old man with a history of gastric cancer showed symptoms like shortness of breath and was found to have lymphadenopathy, pleural effusion, and severe PAH, leading to the diagnosis of AITL through comprehensive testing.
  • The patient responded well to a mini-CHOP chemotherapy treatment, significantly improving his PAH and symptoms, reaching complete remission, highlighting the need to consider AITL in patients presenting with symptoms like PAH.
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  • The study aimed to identify clinical factors affecting survival and treatment outcomes for patients with angioimmunoblastic T-cell lymphoma (AITL) and to create a new prognostic model for this disease.
  • An analysis of 231 AITL patients showed a median age of 63, with most in advanced stages, and the overall survival (OS) and progression-free survival (PFS) were 17 months and 6 months, respectively.
  • The newly developed prognostic model categorizes patients into risk groups based on factors such as age and performance status, allowing for better prediction of survival outcomes, though there's still no clear consensus on the best initial treatment for AITL.
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Unlabelled: To find the independent factors affecting the prognosis of AITL patients, establish a novel predictive model, and stratify the prognosis of AITL patients. We retrospectively analyzed the clinical data of 86 patients diagnosed with AITL in the First Affiliated Hospital of Wenzhou Medical University from December 2010 to March 2022. The clinical features, recurrence time, and death time of patients were collected and analyzed statistically.

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Article Synopsis
  • Angioimmunoblastic T-cell lymphoma (AITL) arises from follicular helper T (Tfh) cells and is difficult to treat due to its resistance to current therapies.
  • The progression of AITL involves a complex interaction between neoplastic Tfh cells and B-cells in the tumor microenvironment, similar to processes in germinal centers.
  • Recurrent mutations like TET2 and DNMT3A in both Tfh and B-cells hint that their relationship could be a potential target for new treatments, highlighting important signaling mechanisms in AITL's pathobiology.
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  • The study aims to compile and summarize published cases of reactive plasmacytosis to assist haematopathologists and clinicians in diagnosing this condition.
  • Researchers reviewed 87 articles covering 146 patients, categorizing cases into six groups, with infectious diseases and angioimmunoblastic T-cell lymphoma (AITL) being the most common causes.
  • The findings emphasize the need for clinicians to consider a wide range of potential diagnoses, especially infections and AITL, when evaluating patients with increased plasma cells in blood or bone marrow.
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Article Synopsis
  • - Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of T-cell lymphoma that can be hard to diagnose because it often looks like other conditions, especially when it presents with skin rashes.
  • - A case study of a 74-year-old man showed how AITL can be misdiagnosed as pure red cell anemia, showing symptoms like direct Coombs-positive anemia and recurrent rashes.
  • - The case highlights the importance of considering lymphoma in patients with specific symptoms and the need for thorough evaluation of medical histories to ensure accurate diagnoses, as early treatment can significantly improve outcomes.
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Laryngeal non-Hodgkin lymphoma: Report of four cases and review of the literature.

Open Life Sci

September 2024

Department of Otolaryngology Head and Neck Surgery, The Affiliated Hospital of Southwest Medical University, No. 25 Taiping Street, Jiangyang District, Luzhou, Sichuan, 646000, China.

Article Synopsis
  • * The first three patients had different types of laryngeal NHL and received various treatments, including surgery, radiotherapy, and chemotherapy.
  • * The authors conclude that further research and more case reports are necessary to better understand and treat this condition.
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Article Synopsis
  • Angioimmunoblastic T cell lymphoma (AITL) is a rare and poorly treated cancer, but a new murine model has been developed to test potential therapies, particularly CAR T cell therapy targeting the cancer-driving CD4 Tfh cells.
  • Researchers utilized a lentiviral vector to create a CD4-specific CAR that only affects CD8 T cells, preventing damage to the CAR T cells themselves.
  • Results showed that these engineered CD8 T cells successfully eliminated malignant CD4 Tfh cells in mouse models, leading to significantly prolonged survival and a reduction in tumor size, indicating a promising new treatment approach for T cell lymphomas.
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  • Chidamide is a targeted histone deacetylase inhibitor that has shown potential in improving the prognosis of angioimmunoblastic T-cell lymphoma (AITL) patients, but real-world outcomes vary regarding its effectiveness on overall survival (OS).
  • A machine learning model was developed using data from 183 AITL patients to predict 2-year OS based on chidamide usage and other clinical features, utilizing techniques like recursive feature elimination and SHAP for interpretability.
  • The study found that the Catboost machine learning model, consisting of 12 important variables, successfully predicted 2-year OS, indicating that incorporating chidamide treatment is associated with improved survival outcomes for AITL patients.
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Article Synopsis
  • Traditional Sanger sequencing struggles with a 5%-20% wild-type mutation detection rate, which isn't sensitive enough for these needs.
  • Newer techniques like Blocker Displacement Amplification (BDA) and others help enhance sensitivity by targeting and blocking wild-type mutations, enabling the detection of low-frequency mutations, such as the RHOA G17V mutation, at 0.5% sensitivity for better MRD monitoring of angioimmunoblastic T-cell lymphoma.
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