Peripheral T-cell lymphomas (PTCLs) are a diverse and uncommon type of lymphoid malignancies with a dismal prognosis. Recent advances in genomic studies have shown recurring mutations that are ...changing our knowledge of the disease's molecular genetics and pathogenesis. As such, new targeted therapies and treatments to improve disease outcomes are currently being explored. In this review, we discussed the current understanding of the nodal PTCL biology with potential therapeutic implications and gave our insights on the promising novel therapies that are currently under study such as immunotherapy, chimeric antigen receptor T-cell therapy, and oncolytic virotherapy.
Objective:
To investigate whether dexmedetomidine (DEX), as adjunctive therapy to benzodiazepine (BZD), is superior to BZD alone in critically ill patients with alcohol withdrawal syndrome (AWS).
...Data Sources:
PubMed Central, Cochrane CENTRAL, ClinicalTrials.gov and Google Scholar were used as search databases. Specific keywords and MeSH terms were “dexmedetomidine,” “benzodiazepine,” and “alcohol withdrawal syndrome.” The last search was on September 16, 2022.
Study Selection and Data Extraction:
Randomized controlled trials (RCTs) and nonrandomized/cohort studies exploring the use of DEX in the management of AWS were included. A total of 12 studies were included in the systematic review and 7 in the meta-analysis.
Data Synthesis:
The intensive care unit length of stay (ICU LOS) was found to have a mean difference (MD) of 48.06 37.48, 58.64, P = <0.001 for the cohort subgroup, significantly favoring the DEX arm, but, in contrast, pooled RCT data showed a result of –20.07 –36.86, –3.28, P = 0.02, a shorter ICU LOS for the DEX arm. Bradycardia and hypotension incidence significantly favored the BZD arm in both subgroups. This study compares the effectiveness of adjunctive DEX in clinical practice and aims to help providers in critical decision-making by compiling and analyzing the best current available evidence of its use in AWS.
Conclusions:
Based on low to very low level of evidence, adjunctive DEX showed no significant difference for ICU LOS when compared with BZD alone. Pooled randomized trials potentially show a benefit but are similarly limited by their low quality of evidence.
Evans syndrome (ES) is a rare autoimmune disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia and autoimmune neutropenia. The precise pathogenesis of ES remains ...unclear, but it is believed to involve immune-mediated destruction of erythrocytes and platelets. Thrombotic complications, such as stroke, are critical yet largely unrecognised in ES. Here, we present a case of an 80-year-old male with ES who developed multiple strokes, emphasising the complex management challenges associated with this condition. The patient, known for stage IIB lung adenocarcinoma, presented with right-sided weakness and was diagnosed with a stroke of undetermined aetiology. He was started on warfarin for secondary prevention alongside intravenous immunoglobulin (IVIG) and corticosteroids for ES. Stroke in ES is rarely reported, and the optimal management remains inconclusive due to its rarity. The patient’s management was guided by existing guidelines for stroke prevention and anticoagulation in the setting of antiphospholipid syndrome. While anticoagulants are recommended for venous thromboembolism prophylaxis in AIHA, there are no clear guidelines for stroke prevention in ES. This case underscores the necessity of individualised treatment approaches and highlights the gaps in evidence regarding stroke management in ES. Future research is essential to determine the optimal management of stroke in this complex clinical scenario.
Blastoid mantle cell lymphoma (MCL) is an extremely rare neoplasm with a dismal prognosis. MCL with an initial presentation in the oral cavity has been rarely reported. This report describes a ...75-year-old male who presented with an oropharyngeal mass causing dysphonia and intermittent hypoxia. A biopsy and immunophenotyping confirmed MCL, favoring the blastoid variant. Imaging showed a 4.2 cm left oropharyngeal polypoid mass with extensive lymphadenopathy. His prognosis was considered unfavorable with elevated Ki-67 index, blastoid morphology, and p53 positivity of malignant cells. There was no central nervous system involvement. He received palliative radiation, resulting in profound tumor reduction and resolution of symptoms. An intensive chemoimmunotherapy was not deemed beneficial due to age, comorbidities, absence of TP53 mutation, and a personal preference for a less aggressive treatment. This case highlights the importance of risk-adapted and personalized management approaches in a very unique presentation of blastoid MCL.
Atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP) was first reported in 1984 as characteristic histological findings in lymph nodes associated with autoimmune diseases, but it has ...not been clearly defined to date. To summarize the histological characteristics and clinical diagnoses associated with ALPIBP, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including “atypical lymphoplasmacytic and immunoblastic lymphadenopathy” from their inception to December 27, 2023. We also summarized the courses of three cases with a pathological diagnosis of ALPIBP. Nine articles with 52 cases were included. Among the total of 55 cases, including the three from our institution, the median age of the cases was 63.5 years with a female predominance (69.5%). Lymphadenopathy was generalized in 65.6% and regional in 34.4% of cases. RA (24.4%), SLE (24.4%), and autoimmune hemolytic anemia (20.0%), were common clinical diagnoses. A combination of cytotoxic chemotherapy was used in 15.6% of cases due to the suspicion of malignancy. Nodal T-follicular helper cell lymphoma, angioimmunoblastic type, methotrexate-associated lymphoproliferative disorders, and IgG4-related diseases were listed as important diseases that need to be pathologically differentiated from ALPIBP. This review summarizes the current understanding of the characteristics of ALPIBP. Given that underrecognition of ALPIBP could lead to overdiagnosis of hematological malignancy and unnecessary treatment, increased awareness of the condition in pathologists and clinicians is crucial.
A 63-year-old male with stage IV hepatocellular carcinoma (HCC), accompanied by lung and adrenal metastases, presented with oral bleeding. Physical examination disclosed bleeding from the tonsillar ...mass. A head and neck computed tomography identified a 2.4 cm enhancing lesion in the right anterior ethmoidal sinus, extending to the nasal region and medial orbit. Tonsillar mass biopsy confirmed HCC metastasis, immunopositive for Hepatocyte Paraffin 1 (HepPar1) and Arginase. He was treated with local radiotherapy (30 fractions). The unique presentation of severe bleeding from a tonsillar biopsy-proven HCC metastatic lesion underscores the rarity of head and neck involvement. Extrahepatic metastasis, particularly to the head and neck area likely due to hematogenous spread, may be a major independent predictor of poor outcomes in HCC patients. Local radiotherapy to achieve local hemostasis and reduce tumor bulk should be considered. In patients with known HCC having new oropharyngeal symptoms, HCC metastasis should be considered for a timely diagnosis. Despite its rarity, this manifestation signifies an unfavorable prognosis, reinforcing the imperative for a multidisciplinary approach to enhance therapeutic outcomes in these complex scenarios.
The central dilemma in treating patients with refractory or relapsed classical Hodgkin lymphoma (RRHL) is the developed resistance to chemotherapy. In recent years, significant advances have been ...made with the introduction of targeted immunotherapy such as brentuximab vedotin (BV) and nivolumab (NV). As monotherapy, BV and NV have demonstrated high response rates but with an opportunity for disease progression. In other studies, BV or NV is given in combination with chemotherapy as a bridge to hematopoietic stem cell transplantation for curative therapy. This review will investigate the effect of BV and NV as single agents, in combination with each other, or given concurrently with chemotherapy on the response and survival rate of patients with RRHL.
Abstract Background Artificial intelligence (AI) has emerged as a tool to potentially increase the efficiency and efficacy of cardiovascular care and improve clinical outcomes. This study aims to ...provide an overview of applications of AI in cardiac surgery. Methods A systematic literature search on AI applications in cardiac surgery from inception to February 2024 was conducted. Articles were then filtered based on the inclusion and exclusion criteria and the risk of bias was assessed. Key findings were then summarized. Results A total of 81 studies were found that reported on AI applications in cardiac surgery. There is a rapid rise in studies since 2020. The most popular machine learning technique was random forest ( n = 48), followed by support vector machine ( n = 33), logistic regression ( n = 32), and eXtreme Gradient Boosting ( n = 31). Most of the studies were on adult patients, conducted in China, and involved procedures such as valvular surgery (24.7%), heart transplant (9.4%), coronary revascularization (11.8%), congenital heart disease surgery (3.5%), and aortic dissection repair (2.4%). Regarding evaluation outcomes, 35 studies examined the performance, 26 studies examined clinician outcomes, and 20 studies examined patient outcomes. Conclusion AI was mainly used to predict complications following cardiac surgeries and improve clinicians' decision‐making by providing better preoperative risk assessment, stratification, and prognostication. While the application of AI in cardiac surgery has greatly progressed in the last decade, further studies need to be conducted to verify accuracy and ensure safety before use in clinical practice.
Introduction: The real-world outcomes in patients (pts) with PCALCL in the era of targeted therapies like Brentuximab Vedotin (BV) is not well defined due to lack of large multicenter studies. We ...conducted a multicenter retrospective RWA to assess patient and disease characteristics, treatment patterns, survival outcomes and impact of novel therapies in this rare type of cutaneous T cell lymphoma (CTCL). Methods: A total of 95 adult (≥18 years) pts from 8 academic institutions in the United States diagnosed from 2010-2022 were included in the analysis. The study was approved by the Institutional Review Boards at the respective sites. Demographic and disease factors were summarized by descriptive statistics. Overall survival (OS) was estimated by Kaplan-Meier method and compared using log rank method. Results: In our cohort, the median age at diagnosis was 63 (range 18-93) years. The majority of pts were male 60%, white 79%, and had good performance status (PS) ECOG 0-1 (93%). Median time from initial presentation to diagnosis was 1.8 (range 0-211) months. Most common initial skin presentation was tumor 46% (44/95), followed by plaques 27% (26/95) and patches 15% (14/95). No pt presented with erythroderma. Median size of the largest lesion was 2 (range 0.5-20) cm. 63% had early stage (IA-IIA) disease and 86% had <5 skin lesions at the time of initial diagnosis. Furthermore, palpable lymph node (LN) (10%) and elevated LDH (18%) were uncommon at diagnosis. Initial treatments include localized radiation 60%, surgical excision 21% and topical steroids 11%. A small subset of pts received systemic treatments like CHOP 13% (12/95), BV 8% (8/95) and oral methotrexate 7% (7/95) as frontline therapy. A proportion of pts received XRT with CHOP (6/12) and BV (2/8). The overall response rate (ORR) to initial therapy was 68% (65/95) with 61% achieving a CR and 17% had primary refractory disease (stable and progressive disease). Relapse rate after 1st line therapy was 52% (49/95). In our cohort a total of 22 pts received BV after frontline therapy, of which 19 were BV naïve and 3 were re-treated. At the time of last follow up, 27% of our cohort were still on treatment, 78% were alive and 21% were dead; only 3% of deaths were attributed to the lymphoma. The median OS was 128 months (95% CI: 78.4-NA) (Figure 1) with a median follow up of 42 (range 0.5-123.5) months. The 5-year OS was 77%. On univariate analysis, older age (≥65 vs <65 years) (median OS 78 vs 128 months; p=0.004) and higher number of lesions (5-10 vs <5) (mOS 34 vs 128 months; p=<0.0001) were significant associated with OS (Table 1). Type and size of the skin lesions, presence of palpable LN, elevated LDH, stage, and treatment modalities had no impact on OS. Results of multivariate analysis will be presented at the conference. Conclusion: In this large multicenter RWA of PCALCL, excellent long-term survival was observed following frontline therapies that were mostly skin directed. The use of systemic therapies including BV were infrequent even in relapsed disease. Older age and increased number of skin lesions were associated with poor OS.
