Hodgkin Lymphoma Presenting as Cardiac Tamponade in a Young Female.

Hodgkin lymphoma (HL) is an uncommon malignancy that is characterized by Hodgkin or Reed-Sternberg cells. Cardiac implications of HL remain one of the least investigated subjects. There are few case reports in the literature of cardiac tamponade in HL patients. We describe a case of a 21-year-old female patient who presented with cardiac tamponade as an initial presentation of HL. Any pericardial effusion significant for tamponade requires immediate drainage and fluid analysis for thorough investigation. Prompt identification and timely intervention are crucial in effectively addressing these complex situations. Therefore, clinicians should maintain heightened awareness in such cases.

Which Is the Best Way to Treat Massive Hemoptysis? A Systematic Review and Meta-Analysis of Observational Studies.

INTRODUCTION: Hemoptysis is one of the most common symptoms of respiratory system diseases. Common causes include bronchiectasis, tumors, tuberculosis, aspergilloma, and cystic fibrosis. The severity of hemoptysis varies from mild to moderate to massive hemoptysis and can easily lead to hemodynamic instability and death from suffocation or shock. Nevertheless, the most threatening hemoptysis that is presented to the emergency department and requires hospitalization is the massive one. In these cases, today, the most common way to manage hemoptysis is bronchial artery embolization (BAE). METHODS: A systematic literature search was conducted in PubMed and Scopus from January 2017 (with the aim of selecting the newest possible reports in the literature) until May 2023 for studies reporting massive hemoptysis. All studies that included technical and clinical success rates of hemoptysis management, as well as rebleeding and mortality rates, were included. A proportional meta-analysis was conducted using a random-effects model. RESULTS: Of the 30 studies included in this systematic review, 26 used bronchial artery embolization as a means of treating hemoptysis, with very high levels of both technical and clinical success (greater than 73.7% and 84.2%, respectively). However, in cases where it was not possible to use bronchial artery embolization, alternative methods were used, such as dual-vessel intervention (80% technical success rate and 66.7% clinical success rate), customized endobronchial silicone blockers (92.3% technical success rate and 92.3% clinical success rate), antifibrinolytic agents (50% clinical success rate), and percutaneous transthoracic embolization (93.1% technical success rate and 88.9% clinical success rate), which all had high success rates apart from antifibrinolytic agents. Of the 2467 patients included in these studies, 341 experienced rebleeding during the follow-up period, while 354 other complications occurred, including chest discomfort, fever, dysphagia, and paresis. A total of 89 patients died after an episode of massive hemoptysis or during the follow-up period. The results of the meta-analysis showed a pooled technical success of bronchial artery embolization equal to 97.22% and a pooled clinical success equal to 92.46%. The pooled recurrence was calculated to be 21.46%, while the mortality was 3.5%. These results confirm the ability of bronchial artery embolization in the treatment of massive hemoptysis but also emphasize the high rate of recurrence following the intervention, as well as the risk of death. CONCLUSION: In conclusion, massive hemoptysis can be treated with great clinical and technical success using bronchial artery embolization, reducing mortality. Mortality has now been reduced to a small percentage of cases.

Assessment of Atherosclerosis in Ischemic Stroke by means of Ultrasound of Extracranial/Intracranial Circulation and Serum, Urine, and Tissue Biomarkers.

It is a common practice to take into consideration age, diabetes, smoking, treated and untreated systolic blood pressure, total cholesterol, and high-density lipoprotein cholesterol for the prediction of atherosclerosis and stroke. There are, however, ultrasound markers in use for the assessment of atherosclerosis and the evaluation of stroke risk. Two areas of investigation are of interest: the carotid artery and the intracranial arterial circulation. Again, within the domain of the carotid artery, two ultrasonic markers have attracted our attention: intima media thickness of the carotid artery and the presence of carotid plaque with its various focal characteristics. In the domain of intracranial circulation, the presence of arterial stenosis and the recruitment of collaterals are considered significant ultrasonic markers for the above-mentioned purpose. On the other hand, a series of serum, urine, and tissue biomarkers are found to be related to atherosclerotic disease. Future studies might address the issue of whether the addition of proven ultrasonic carotid indices to the aforementioned serum, urine, and tissue biomarkers could provide the vascular specialist with a better assessment of the atherosclerotic load and solidify their position as surrogate markers for the evaluation of atherosclerosis and stroke risk.

Adenoviral Infections in Neonates: A Case-Based Literature Review.

Adenoviral infections in neonates are associated with high rates of mortality due to the lack of humoral immunity. A comprehensive search of published literature in PubMed, Google Scholar, and Science Direct electronic databases was conducted for case reports published between the years 1990 and 2021. The aim of our study is to investigate the risk factors, clinical manifestations, treatment, and outcomes of adenoviral infections in neonates. In our study, 36 cases were included. The most common type of infection was disseminated one (14/36, 38.8%), followed by adenoviral pneumonia (13/36, 36.1%). Cidofovir was administered in seven cases (19.4%), and death was reported in six of them. One preterm low birthweight neonate with disseminated adenoviral infection was treated with a combination of cidofovir, intravenous immune globulin, and haploidentical virus-specific T lymphocytes (VSTs) and survived. In this review, we found a statistically significant difference in the outcome based on the type of adenoviral infection (p=0.001). Disseminated infection and pneumonia are associated with the worst prognosis. In addition, mortality was observed to be higher in neonates with disseminated disease in contrast to neonates with localized infection (p=0.002). However, the antiviral treatment had no statistically significant effect on the mortality rate (p=0.137). There is a necessity for further investigation and randomized studies to validate the results of the present study.

Parvovirus B19-Associated Myocarditis: A Literature Review of Pediatric Cases.

Parvovirus B19 (B19V) infection may lead to myocarditis, a life-threatening condition in pediatric patients. In this review, we aim to present published pediatric cases of B19V-associated myocarditis in order to understand the deep complex connections and draw useful conclusions. We performed a comprehensive search of MEDLINE, Science Direct, and Google Scholar electronic databases. A total of 32 cases were included in our study. The most common presenting symptom was tachycardia in 22/32 patients (68.7%), followed by tachypnoea (21/32, 65.6%), fever, and rash (12/32, 37.5% for both of them). Cardiac arrest, loss of consciousness, and systemic infection were associated with the worst prognosis, with statistically significant differences (p-value 0.001, 0.02, 0.001. respectively). A percentage as high as 90.4% of patients with left ventricular (LV) dysfunction and reduced ejection fraction (EF) were discharged. Twelve patients required ventilatory support, five required extracorporeal membrane oxygenation (ECMO), and three underwent heart surgery. Treatment with immunosuppressive agents and immunoglobulin was found to be beneficial for patients (p-value 0.006 and 0.004, respectively). In conclusion, B19V myocarditis has high mortality rates in children. There is no specific antiviral treatment for B19V infection and therapeutic strategies for myocarditis aim to delay the worsening of heart failure and to preserve the LV function. Inotropic drugs, diuresis, ventilatory support, Intravenous immunoglobulin (IVIG), and immunosuppressive therapy seem to help the recovery of the myocardium in children with LV dilation, dysfunction, and reduced EF. Children with cardiac arrest, arrhythmias, and loss of consciousness have the worst prognosis.

Acute leukemia presenting as acute lower limb ischemia.

Objectives: Acute lower limb ischemia (ALLI) is a common vascular emergency. However, ALLI presenting as the initial symptom of acute leukemia (AL) is scarce. Here we present a case of ALLI in the setting of acute myeloid leukemia (AML) while systematically reviewing the current literature to withdraw conclusions about the management, prognosis, and treatment for this atypical presentation of AL. Methods: We conducted a systematic electronic research according to Preferred Reporting Items for Systematic Review and Meta-Analysis protocol (PRISMA) for articles published from January 1981 up to January 2021 concerning ALLI in the setting of acute leukemia (AL). Patients' baseline characteristics were recorded and nine outcomes of interest were studied. Results: Twenty-six individuals, 16 males with a mean age of 46.3 years (±20) were included in this review. The diagnosis included 13 AML patients (50%), 11 acute promyelotic leukemia (APL) (42.3%) and two acute lymphoblastic leukemias (ALL) (7.7%). Treatment varied among nine different regimens. Four patients were treated with chemotherapy alone (15.4%), four with thrombectomy alone (15.4%), and 11 with a combination of chemotherapy and thrombectomy (42.3%). Eight major amputations were recorded (30. 8%). Thirty-day mortality was 35.7%. Forty-eight peripheral thrombotic events were recorded with 12 patients suffering recurrent thrombotic events. Conclusion: ALLI as the presenting symptom of AL is a rare condition that carries significant mortality and amputation rates. Timely diagnosis is crucial concerning short-term survival and limb salvage. APL, despite being the rarest form of AL, represented a significant proportion of the patient population in this review. The role of leukostasis in the disease's progression and the efficacy of leukapheresis as a treatment regimen should be further investigated through case-control studies.

Tixagevimab/Cilgavimab in SARS-CoV-2 Prophylaxis and Therapy: A Comprehensive Review of Clinical Experience.

Effective treatments and vaccines against COVID-19 used in clinical practice have made a positive impact on controlling the spread of the pandemic, where they are available. Nevertheless, even if fully vaccinated, immunocompromised patients still remain at high risk of adverse outcomes. This has driven the largely expanding field of monoclonal antibodies, with variable results. Tixagevimab/Cilgavimab (AZD7442), a long-acting antibody combination that inhibits the attachment of the SARS-CoV-2 spike protein to the surface of cells, has proved promising in reducing the incidence of symptomatic COVID-19 or death in high-risk individuals without major adverse events when given as prophylaxis, as well as early treatment. Real-world data confirm the antibody combination's prophylaxis efficacy in lowering the incidence, hospitalization, and mortality associated with COVID-19 in solid organ transplant recipients, patients with immune-mediated inflammatory diseases and hematological malignancies, and patients in B-cell-depleting therapies. Data suggest a difference in neutralization efficiency between the SARS-CoV-2 subtypes in favor of the BA.2 over the BA.1. In treating COVID-19, AZD7442 showed a significant reduction in severe COVID-19 cases and mortality when given early in the course of disease, and within 5 days of symptom onset, without being associated with severe adverse events, even when it is used in addition to standard care. The possibility of the development of spike-protein mutations that resist monoclonal antibodies has been reported; therefore, increased vigilance is required in view of the evolving variants. AZD7442 may be a powerful ally in preventing COVID-19 and the mortality associated with it in high-risk individuals. Further research is required to include more high-risk groups and assess the concerns limiting its use, along the SARS-CoV-2 evolutionary trajectory.

Adenoviral Infections in Bone Marrow Transplanted Adult Patients: A Review of the 44 Cases Reported in the Last 25 Years.

Adenoviral infections in immunocompromised individuals may be life-threatening conditions. The aim of this review is to document all the reported cases of adenoviral infection is patients having undergone bone marrow transplantation (BMT). A comprehensive literature search of the databases Pubmed, Science Direct, and Google Scholar was conducted to identify all the case reports of adenoviral infections in BMT patients. A total of 30 articles with 44 patients were included. The most common underlying condition was acute lymphocytic leukemia (23%) followed by acute myeloid leukemia (18%). The most common site of infection was disseminated (50%), followed by liver infection (8%) and hemorrhagic cystitis (8%). Cidofovir was administered in 40.9% of the cases, and death was reported in 34.4% of them. Ribavirin was administered as monotherapy in 15.9% of patients, with a mortality rate of 57.1%. We found that the antiviral drug option had no statistically significant effect on the mortality rate (p=0.242). Also, the absence of graft-versus-host disease (GVHD) was not associated with an improved outcome (p=0.523). There was, however, a statistically significant difference in the outcome based on the site of infection (p=0.005), with a higher rate of mortality in the disseminated and gastrointestinal cases. To the best of our knowledge, this is the first review documenting all the cases of adenoviral infections in BMT patients. Future randomized studies are needed to validate the results of the present study.

Does the COVID-19 Pandemic Affect Morbidity and Mortality Rates of Emergency General Surgery? A Retrospective Study from a Single-Center Tertiary Greek Hospital.

Background and Objectives: The outbreak of the COVID-19 pandemic had a major impact on all aspects of health care. Few up-to-date studies have actually assessed the impact of COVID-19 on emergency surgeries. The aim of this study was to provide an overview of the impact of the pandemic relating to the emergency surgery performed, as well as morbidity and mortality rates during the first year of the pandemic (March 2020-February 2021) and during the control period. In this period, the first propaedeutic surgery department and the third surgery department of the University General Hospital of Thessaloniki "AHEPA" in Greece provided continuous emergency general surgery services. Material and Methods: The study is in a retrospective cohort and included patients who were admitted to the Emergency Department and underwent emergency general surgery during the control period (n = 456), March 2019-February 2020 and during the first year of the pandemic (n = 223), March 2020-February 2021. Gender, age, type of surgical operation (morbidity), ICU need, the patient's outcome, and days of hospitalization were compared. Results: A total of 679 emergency surgeries were included. Statistically significant differences emerged between the two time periods in the total number of emergency surgeries performed (p < 0.001). The most common type of surgery in the control period was associated with soft tissue infection while, during the pandemic period, the most common type of surgery was associated with the hepatobiliary system. In addition, the mortality rates nearly doubled during the pandemic period (2.2% vs. 4%). Finally, the mean age of our sample was 50.6 ± 17.5 and the majority of the participants in both time periods were males. Conclusions: The COVID-19 pandemic changed significantly the total number of emergency general surgeries performed. Mortality rates doubled and morbidity rates were affected between the control and pandemic periods. Finally, age, gender, length of hospitalization, intensive care unit hospitalization, and laparoscopy use in patients undergoing emergency surgery during the pandemic were stable.

Diagnosing a Patient with Erdheim-Chester Disease during the COVID-19 Pandemic.

Background: Erdheim-Chester disease (ECD) is a rare hematopoietic neoplasm of histiocytic origin characterized by an insidious course. The coronavirus disease 2019 (COVID-19) pandemic has put an enormous strain on healthcare systems worldwide both directly and indirectly, resulting in the disruption of healthcare services to prevent, diagnose and manage non-COVID-19 disease. Case Presentation: We describe the case of a 58-year-old male patient with sporadic episodes of self-resolving mild fever and anemia of chronic disease with onset two years before the current presentation. Positron emission/computed tomography scan revealed the presence of moderately hypermetabolic perirenal tissue masses. In order to achieve diagnosis, repeated perirenal tissue biopsies were performed, and the diagnostic evaluation was complicated by the strain put on the healthcare system by the COVID-19 pandemic. The patient contracted SARS-CoV-2 and required hospitalization, but recovered fully. No further ECD target organ involvement was documented. Treatment options were presented, but the patient chose to defer treatment for ECD. Conclusion: A high index of suspicion and multidisciplinary team collaboration is paramount to achieve diagnosis in rare conditions such as ECD. Disruptions in healthcare services in the pandemic milieu may disproportionately affect people with rare diseases and further study and effort is required to better meet their needs in the pandemic setting.

Effectiveness and Safety of Romiplostim Among Patients with Newly Diagnosed, Persistent and Chronic Immune Thrombocytopenia in European Clinical Practice.

INTRODUCTION: Romiplostim has been approved in Europe since 2009 to treat patients with chronic primary immune thrombocytopenia (ITP). Using real-world data from seven European countries, we measured the effectiveness and safety outcomes within 24 weeks following romiplostim initiation by duration of ITP: less than 3 months ("newly diagnosed"), 3-12 months ("persistent"), and more than 12 months ("chronic"). METHODS: Adults with ITP and ≥ 1 romiplostim administration between 2009 and 2012 were included. Endpoints included durable platelet response, median platelet count, rescue therapy, bleeding and adverse events. We used inverse probability of censoring weighted estimators to estimate cumulative risk of each outcome. There were 64 newly diagnosed, 50 persistent, and 226 chronic ITP patients at romiplostim initiation. RESULTS: Durable platelet response at 24 weeks ranged from 32% [confidence interval (CI): 18-46%] in newly diagnosed patients to 53% (CI 37-68%) in persistent patients. Median platelet count during follow-up ranged from 88 (CI 80-96) × 109/L in chronic patients to 131 (CI 102-160) × 109/L in newly diagnosed patients. CONCLUSION: Regardless of ITP duration, over half of patients discontinued concomitant ITP medications. Few adverse events were observed. Although only approved for chronic patients, estimates of the romiplostim treatment effect were similar across patients being managed in European clinical practice, regardless of ITP duration at romiplostim initiation.

Dasatinib associated lymphadenopathy in a chronic myeloid leukemia patient: A case report.

RATIONALE: Dasatinib associated lymphadenopathy (DAL) is a rare adverse event in chronic myeloid leukemia patients (CML). A case of voluminous lymphadenopathy in the context of DAL is presented. PATIENT CONCERNS: A 40-year-old male patient was diagnosed with BCR-ABL1 positive chronic stage CML 2 years ago and achieved complete molecular response on nilotinib, which was switched to dasatinib due to nilotinib intolerance. After 5 months on dasatinib, the patient presented with a large mass in the axillary region. DIAGNOSIS: Common infectious and autoimmune etiologies of lymphadenopathy were ruled out. The positron emission tomography/computed tomography (PET/CT) demonstrated a hypermetabolic lymphadenopathy highly suspicious of lymphoma. The subsequent biopsy excluded lymphoma or extramedullary blastic transformation of CML and revealed reactive lymphadenopathy with mixed (cortical and paracortical) pattern. Clinical history and clinicopathological correlation suggested the diagnosis of DAL. INTERVENTION: Dasatinib was discontinued and the patient remained in close follow-up. TKI treatment with nilotinib was reinitiated. OUTCOMES: Lymphadenopathy resolved clinically at 4 weeks and normalization of PET/CT findings was documented at 9 weeks after cessation of the drug. TKI treatment with nilotinib was reinitiated with good tolerance. LESSONS: DAL may present with voluminous lymphadenopathy consistent with malignancy in clinical and imaging workup. We describe the spectrum of lesions associated with DAL and identify common features with drug-induced lymphadenopathy.

Systemic lupus erythematosus in a patient with multiple system atrophy: A case report.

RATIONALE: Multiple system atrophy is a late-onset rare neurodegenerative movement disorder which results in debilitating disease. Fever frequently ensues in the context of infections which can be associated with significant morbidity and mortality, but among alternative diagnostic possibilities neoplasms and autoimmune disorders should be considered. PATIENT CONCERNS: We describe a case of a prolonged febrile syndrome in a 55-year-old female patient with onset of multiple system atrophy two years before presentation. Patient history and symptoms were not contributive to guide the diagnostic work-up. DIAGNOSIS: Initial evaluation provided no specific findings. Repeat testing of auto-antibodies revealed positive antinuclear and anti-ds DNA antibodies coupled with low complement which in conjunction with renal biopsy substantiated the diagnosis of systemic lupus erythematosus flare. INTERVENTION: Pending the biopsy result, treatment with hydroxychloroquine and corticosteroids was initiated. Due to failure to achieve remission, azathioprine was added, but symptoms persisted. Following the diagnosis of lupus nephritis, azathioprine was discontinued and induction treatment with cyclophosphamide in accordance with the Euro-Lupus regimen was initiated and upon completion followed by maintenance therapy with mycophenolate mofetil. OUTCOMES: The patient achieved remission after cyclophosphamide was added to treatment with corticosteroids and has not experienced new flares during the next two years. The neurological syndrome has remained stable during this period. LESSONS: To our knowledge, we report the first case of concurrent systemic lupus erythematosus and multiple system atrophy. Prolonged fever presents unique challenges in patients with rare diseases.

Assessment of Self-Administration of Romiplostim in Patients with Immune Thrombocytopenic Purpura after Receipt of Home Administration Training Materials: a Cross-Sectional Study.

INTRODUCTION: Romiplostim is a subcutaneously administered thrombopoietin-receptor agonist approved in the European Union for self-administration (or administration by a caregiver) in selected adult patients with chronic primary immune thrombocytopenia refractory to other treatments. To mitigate the risk of medication errors due to self-administration, the manufacturer has implemented additional risk minimisation measures (RMM) in the form of a Home Administration Training (HAT) pack to support the training of both healthcare professionals (HCPs) (guide and checklist for patient selection and training) and patients (a preparation mat, quick guide booklet, step-by-step guide, self-administration diary and DVD/video). OBJECTIVE: The primary objective was to estimate the proportion of patients/caregivers who administered romiplostim correctly after HAT pack training. METHODS: A multicentre observational study was conducted to evaluate the effectiveness of the HAT pack by recording data on a standardised collection form during direct observation of patients/caregivers in the act of administering romiplostim at the first standard-of-care visit 4 weeks after training with the HAT pack. RESULTS: Among the 40 patients/caregivers enrolled across 12 study centres in eight European countries, 35 [87.5%; 95% confidence interval (CI) 73.9-94.5] administered romiplostim correctly, and five (12.5%; 95% CI 5.5-26.1) did not. CONCLUSION: The correct administration of romiplostim by most patients/caregivers supports the effectiveness of the HAT pack as an additional risk minimisation tool in the population and setting of this study.

Real-world data on prognosis and outcome of primary plasma cell leukemia in the era of novel agents: a multicenter national study by the Greek Myeloma Study Group.

We have studied the efficacy and the prognostic impact of novel agents in 50 primary plasma cell leukemia (pPCL) patients registered in our database. Eighty percent of patients were treated upfront with novel agent-based combinations; 40% underwent autologous stem cell transplantation (ASCT). Objective response rate was 76; 38% achieved at least very good partial response (≥vgPR) and this correlated significantly with bortezomib-based therapy plus ASCT. At the time of evaluation, 40 patients had died. Early mortality rate (≤1 month) was 6%. Median progression-free survival (PFS) and overall survival (OS) were 12 months and 18 months respectively, both significantly longer in patients treated with bortezomib-based therapy + ASCT vs. others (PFS: 18 vs. 9 months; p = 0.004, OS: 48 vs. 14 months; p = 0.007). Bortezomib-based therapy + ASCT predicted for OS in univariate analysis. In multivariate analysis, achievement of ≥vgPR and LDH ≥ 300 U/L were significant predictors for OS. These real-world data, based on one of the largest reported national multicenter series of pPCL patients treated mostly with novel agents support that, among the currently approved induction therapies, bortezomib-based regimens are highly effective and reduce the rate of early mortality whereas in combination with ASCT consolidation they prolong OS.

CD5+ B lymphoproliferative disorder with subsequent development of plasma cell leukaemia: Diagnostic and aetiologic reasoning.

BACKGROUND: Plasma cell myeloma (PCM) has been sporadically reported to occur simultaneously or subsequently to mature B lymphoproliferative disorders (LPDs), predominantly chronic lymphocytic leukaemia (CLL). METHODS: We describe the clinical and laboratory findings of a 69-year-old male patient who developed plasma cell leukaemia (PCL) 8 years after an initial diagnosis of a low stage CD5+ B LPD and 3 years after treatment for LPD. RESULTS: The transition from a clinically indolent B LPD to an aggressive PCM was documented by bone marrow (BM) biopsy, while flow cytometric (FC) immunophenotyping conferred additional information by disclosing the co-existence of both disorders in BM and the presence of abnormal monotypic PCs in peripheral blood above PCL levels. Phenotypic findings suggested a discrete clonal origin of the two disorders. CONCLUSIONS: This report of PCL development in a patient with residual CD5+ B LPD, emphasizes the need for comprehensive diagnostic evaluation of such cases and scrutiny of their aetiological relationship, including FC immunophenotyping due to its high analytical sensitivity and multiparametric capacity compared to morphology or immunohistochemistry alone. © 2017 International Clinical Cytometry Society.