Severe Fatal ARDS Due to Untreated Human Granulocytic Anaplasmosis in a 67-Year-Old Man: A Case Report.

BACKGROUND Anaplasmosis, or human granulocytic anaplasmosis (HGA), is a tick-borne diseased caused by a gram-negative, intracellular bacterium, Anaplasma phagocytophilum. HGA usually presents with mild symptoms but can be more severe. This report describes a 67-year-old male resident of rural Pennsylvania, admitted to the hospital after a fall, who developed fatal acute respiratory distress syndrome (ARDS) associated with human granulocytic anaplasmosis (HGA) following transmission of Anaplasma phagocytophilum by a tick bite (Ixodes scapularis). CASE REPORT A 67-year-old man, resident of rural Pennsylvania, with history of diabetes mellitus, presented after falling from a 7-foot-tall ladder, sustaining right-sided hemopneumothorax, multiple right rib fractures, and unstable T12 vertebra fracture. He required tube thoracostomy and underwent T9-L2 posterior spinal fusion surgery. His initial labs showed leukopenia, thrombocytopenia, and elevated transaminase levels. His course was complicated by cardiac arrest and acute respiratory failure, consistent with severe ARDS. He received high positive end-expiratory pressure (PEEP) ventilation, prone positioning, and neuromuscular paralysis to improve refractory hypoxemia. Bronchoalveolar lavage (BAL) for bacterial, fungal, viral pathogens, Covid-19, respiratory viral panel, Mycoplasma pneumoniae, and Chlamydia pneumoniae were negative. his family withdrew medical care, knowing the patient's own wishes, and the patient died. Polymerase chain reaction (PCR) for Anaplasma DNA came back positive after the patient's death. His peripheral smear was then examined, showing morulae inside the cytoplasm of infected neutrophils.   CONCLUSIONS This report describes the atypical presentation of a case of HGA and highlights that in parts of the world where tick-borne diseases are endemic, disease awareness, high index of clinical suspicion, and early diagnosis and management are required.

Gastrointestinal endoscopy 30-day-associated bacteremia: Nonoutbreak 5-year review in an inner-city, tertiary-care hospital.

BACKGROUND: Gastrointestinal endoscopic procedures (GIEP's) are an essential part of patient care both diagnostically and therapeutically. Post-GIEP infections may be higher than previously reported and may not have been accurately captured in the past. The aim of this study was to determine the incidence and associated factors of bacteremia associated with GIEP's. METHODS: This is retrospective study of GIEPs performed over a five-year period (2018-2022) at an academic medical center. Electronic health records (EHR) identified GIEPs and positive blood cultures within 30 days of procedure. Statistical analysis was performed using non-parametric testing to compare variables due to the small number of positive blood cultures. RESULTS: EHR identified 18,986 GIEP's and 52 true and unique bacteremia out of 17,093 blood cultures during the five-year study period. The highest rate of positive blood culture of 2.84% (18/ 634) was associated with ERCP and the lowest 0.08% (7/ 9029) was associated with colonoscopy. DISCUSSION: Our study showed a reflection of the endemic rate of bacteremia post GIEP's. Our study cannot differentiate endogenous infection versus contaminated (exogenous) endoscopes. ERCP procedures are disproportionately associated with higher incidence of bacteremia. CONCLUSIONS: Clinical surveillance in non-outbreak settings is essential for estimating GIEP related infections. It should be combined with endoscopic reprocessing audits for appropriate prevention of GIEP associated infections.

Chronic Myeloid Leukemia in Adolescents and Young Adults: Clinicopathological Variables and Outcomes.

INTRODUCTION: Adolescents and young adults (AYAs) diagnosed with chronic myeloid leukemia (CML) constitute a significant demographic group, particularly in regions with youthful populations like Qatar. Despite the global median age of CML diagnosis being 65 years, Qatar's age distribution reflects a younger cohort. This study investigates whether AYAs with CML exhibit distinct clinicopathological characteristics or outcomes compared to older age groups. METHODS: A total of 224 CML patients were enrolled, including 114 AYAs (defined as ages 15 through 39). Demographic and clinical parameters, including gender, BMI, BCR-ABL1 transcript type, white blood cell (WBC) count, hemoglobin level, platelet count, and spleen size, were compared between AYAs and older patients. Prognostic scoring systems (Sokal, Hasford, EUTOS, and ELTS) and molecular response rates (MMR and DMR) were also evaluated. RESULTS: AYAs demonstrated higher WBC counts at diagnosis (median 142.3 vs. 120; p = 0.037) and lower hemoglobin levels (10.5 vs. 11.40; p = 0.004) compared to older patients. Spleen size was significantly larger in AYAs (18.8 vs. 15.5; p = 0.001). While AYAs showed better prognostic scores by Sokal and Hasford criteria, EUTOS and ELTS scores indicated comparable risk stratification. However, AYAs exhibited lower rates of MMR (56.7% vs. 73.4%; p = 0.016) and achieved MMR at a slower pace (median time 130 vs. 103 months; p = 0.064). Similarly, the percentage of DMR was lower in AYAs (37.1% vs. 46.8%; p = 0.175). CONCLUSION: Despite their younger age, AYAs with CML displayed poorer prognoses compared to older patients. These findings underscore the importance of tailored management strategies for AYAs with CML to optimize outcomes in this distinct patient population.

The Safety of Novel Therapies in Chronic Lymphocytic Leukemia in the Era of Intermittent Fasting: A Pharmacology-Based Review.

Intermittent fasting (IF) has recently gained popularity due to its emerging benefits in reducing weight and improving metabolic health. Concurrently, novel agents (NAs) like venetoclax and Bruton tyrosine kinase inhibitors (BTKIs) have revolutionized the treatment of chronic lymphocytic leukemia (CLL). Unfortunately, it is unclear whether the associated risks of tumor lysis syndrome (TLS) and gastrointestinal bleeding (GIB) are increased in IF practitioners receiving NAs. This review explored the literature available on the permissibility of IF in CLL patients undergoing treatment with first-line NAs (FLNAs). Literature was scoped to identify IF patterns and the available data on TLS and GIB risks associated with food and fluid intake in CLL patients receiving FLNAs. Although current evidence is insufficient to recommend IF in this population, it may be possible for patients on venetoclax to conservatively practice fluid-liberal IF, provided that adequate hydration and the consistent administration of food are achieved. In contrast, considering the significant risk of TLS and the pharmacokinetics of venetoclax, patients should be discouraged from practicing fluid-restricted IF, especially during the ramp-up phase. Moreover, patients on BTKIs ought to refrain from IF due to the possible risk of GIB until further data are available. Further research is needed to provide conclusive recommendations.

Current Status and Management of Chronic Myeloid Leukemia in the Gulf Region: Survey Results and Expert Opinion.

Studies on chronic myeloid leukemia (CML) in the Gulf region are scarce, consisting of a survey and expert meeting that included 15 experts in 2023 which discussed CML diagnosis, testing, treatment objectives, toxicities, and discontinuation in the Gulf region. Most patients were reported to be in first-line therapy, and the most common treatments were imatinib/imatinib generic in first-line and dasatinib in second- and third-lines. Mutation analysis was not reported to be routinely performed at the time of diagnosis but rather in case of progression to accelerated/blast phase or any sign of loss of response. While all participants were aware that BCR-ABL should be monitored every three months during the first year of treatment, 10% reported monitoring BCR-ABL every six months in practice due to test cost and lab capability. The most important first-line therapy objective was "achievement of major molecular response" (MMR) in younger patients and "overall survival" in older ones. The most important treatment objectives were "MMR" and "early molecular response followed by prolongation of overall survival" in the short term and "treatment-free remission" in the long term. The current practices in CML in the Gulf region appear to be similar to global figures.

Health-related quality of life in adults with hematological cancer: a 2023 cross-sectional survey from Qatar.

Background: Hematological cancers impose a complex burden on individuals, affecting their physical health and mental and emotional well-being. This study evaluated the health-related Quality of Life (HRQOL) and its determinants among adults with hematological cancers in Qatar in 2023. Methods: A cross-sectional study used a validated structured questionnaire conducted among adult patients diagnosed with hematological cancers. All adult patients who attended The National Centre for Cancer Care and Research (NCCCR) in Qatar during the Data collection period (January to March 2023) and agreed to participate were included in the study. Results: A total of 257 participants were enrolled in the study. The highest median (IQR) score of the HRQOL domain was observed in the functionating score of 90.6 (13.8), followed by the global health score of 83.3(25. 0). The median (IQR) of the symptoms burden score was 07.4(12.3). Gender significantly affects HRQOL, with males reporting better functioning and lower symptom burden than females. Employment status is positively associated with functioning scores. Regular exercise correlates with higher global health and functioning scores and lower symptom burden, while depressive symptoms are linked to poorer HRQOL outcomes. Patients experiencing cancer recurrence or active disease report lower global health and functioning scores and higher symptom burden. Treatment modalities such as chemotherapy and bone marrow transplant (BMT) timing also influence HRQOL, with recent treatment recipients showing lower global health and higher symptom burden scores. Depressive symptoms were the primary factor, lowering the global health score by 15.2%. Regarding the low functioning score predictors, female gender, depressive symptoms, and cancer recurrence emerged as significant predictors of the low functioning score. Furthermore, Regular exercise increased the functioning score by 03.4 units (p-value=0.018). Finally, Multiple linear regression analysis reinforced the significance of depressive symptoms, active disease status, and recurrence within the past five years as substantial predictors of higher symptom scores. Conclusions: The study emphasizes the profound impact of depressive symptoms on all aspects of Health-Related Quality of Life (HRQOL), mainly affecting global health. It highlights the positive role of regular exercise in enhancing global health, functioning, and symptom burden scores.

Nickel augmented biochar for sustaining produced water treatment to decarbonize oil and gas industrial waste using anaerobic-aerobic granular cylindrical periodic discontinuous batch reactors.

This study assessed the efficacy of granular cylindrical periodic discontinuous batch reactors (GC-PDBRs) for produced water (PW) treatment by employing eggshell and waste activated sludge (WAS) derived Nickel (Ni) augmented biochar. The synthesized biochar was magnetized to further enhance its contribution towards achieving carbon neutrality due to carbon negative nature, Carbon dioxide (CO2) sorption, and negative priming effects. The GC-PDBR1 and GC-PDBR2 process variables were optimized by the application of central composite design (CCD). This is to maximize the decarbonization rate. Results showed that the systems could reduce total phosphorus (TP) and chemical oxygen demand (COD) by 76-80% and 92-99%, respectively. Optimal organic matter and nutrient removals were achieved at 80% volumetric exchange ratio (VER), 5 min settling time and 3000 mg/L mixed liquor suspended solids (MLSS) concentration with desirability values of 0.811 and 0.954 for GC-PDBR1 and GC-PDBR2, respectively. Employing four distinct models, the biokinetic coefficients of the GC-PDBRs treating PW were calculated. The findings indicated that First order (0.0758-0.5365) and Monod models (0.8652-0.9925) have relatively low R2 values. However, the Grau Second-order model and Modified Stover-Kincannon model have high R2 values. This shows that, the Grau Second Order and Modified Stover-Kincannon models under various VER, settling time, and MLSS circumstances, are more suited to explain the removal of pollutants in the GC-PDBRs. Microbiological evaluation demonstrated that a high VER caused notable rises in the quantity of several microorganisms. Under high biological selective pressure, GC-PDBR2 demonstrated a greater percentage of nitrogen removal via autotrophic denitrification and a greater number of nitrifying bacteria. The overgrowth of bacteria such as Actinobacteriota spp. Bacteroidota spp, Gammaproteobacteria, Desulfuromonas Mesotoga in the phylum, class, and genus, has positively impacted on granule formation and stability. Taken together, our study through the introduction of intermittent aeration GC-PDBR systems with added magnetized waste derived biochar, is an innovative approach for simultaneous aerobic sludge granulation and PW treatment, thereby providing valuable contributions in the journey toward achieving decarbonization, carbon neutrality and sustainable development goals (SDGs).

The Impact of Prior Authorization on Clinical Practice and Patient Care Outcomes: A Work Group Report of the AAAAI Prior Authorization Task Force.

The Prior Authorization Task Force of the American Academy of Allergy, Asthma & Immunology (AAAAI), a presidential initiative of David Khan, MD, FAAAI, was established to develop an AAAAI position statement outlining ways to improve health care for our patients, to support legislation that advocates for prior authorization (PA) reform and identify the impact PA has on its membership using a questionnaire survey. This article describes the results of this survey. An electronic anonymous survey questionnaire was developed to assess the impact and burden of PA on AAAAI members and their staff and patients. Surveys were sent to randomly selected members and fellows of the AAAAI in the United States. Descriptive statistics were used to analyze the results by the Information Services team of the AAAAI and the authors of this work group report. The questionnaire responses from allergy immunology specialists demographically reflected the AAAAI membership and indicate that PAs can significantly affect patient care delivery and increase administrative burden to clinical practices, leading to serious adverse events in some circumstances. Differential responses regarding PAs for various medication classes likely reflect the physician's patient population, which can shift prescribing patterns. Prior authorization is a serious health care problem that is wasting financial resources and needlessly placing patients in danger when they are unable to access medications or medical services required for clinical management. The results of this questionnaire study support the recommendations made in the recent AAAAI position statement on PA.

CAR-T cell therapy: Efficacy in management of cancers, adverse effects, dose-limiting toxicities and long-term follow up.

Chimeric Antigen Receptor T-cell (CAR-T) therapy has emerged as a groundbreaking and highly promising approach for the management of cancer. This paper reviews the efficacy of CAR-T therapy in the treatment of various hematological malignancies, also, with a mention of its effect on solid tumors, for which they have not received FDA approval yet. Different common and uncommon side effects are also discussed in this paper, with attention to the effect of each drug separately. By reviewing the recommendations of the FDA for CAR-T therapy research, we have extensively discussed dose-limiting toxicities. This further highlights the need for precise dosing strategies, striking a balance between therapeutic benefits and potential risks. Additionally, we reviewed the long-term follow-up of patients receiving CAR-T therapy to gain valuable insights into response durability and late-onset effects.

Myocardial Infarction in Chronic Myeloid Leukemia: Results from the Nationwide Readmission Database.

INTRODUCTION: Chronic myeloid leukemia (CML) is a hematological malignancy with an excellent prognostic outcome. After the advancements in CML treatment and the introduction of different tyrosine kinase inhibitors (TKIs), the life expectancy of CML patients has become equivalent to that of the general population. As a result, coronary artery disease is anticipated to be the leading cause of death among CML patients. Moreover, TKI use is associated with a risk of endothelial dysfunction, thrombosis, and cardiovascular events, including myocardial infarction. In this study, we compare the outcomes of percutaneous coronary intervention (PCI) in patients with CML to their matched non-CML counterparts. METHOD: This is a retrospective cohort study using the Nationwide Readmission Database from January 2016 to December 2020. Adults with or without CML hospitalized for acute myocardial infarction and underwent PCI were included. The patients were identified using ICD-10 codes. The primary outcomes were in-hospital mortality and 30-day readmission rates. The secondary outcomes were PCI complications rates. RESULTS: Out of 2,727,619 patients with myocardial infarction, 2,124 CML patients were identified. A total of 888 CML patients underwent PCI. CML patients were significantly older (mean age: 68.34 ± 11.14 vs. 64.40 ± 12.61 years, p < 0.001) than non-CML patients without a difference in sex distribution. Hypertension (85.45% vs. 78.64%), diabetes (45.48% vs. 37.29), stroke (11.84% vs. 7.78) at baseline were significantly higher in the CML group. Prior myocardial infarction events (20.51% vs. 15.17%) and prior PCI procedure (24.47% vs. 16.89%) were significantly higher in the CML group. CML patients had a significantly longer hospital stay (4.66 ± 4.40 vs. 3.75 ± 4.62 days, p = 0.001). The primary outcomes did not differ between the comparison groups. The risk of post-PCI complications did not differ between the comparison groups in the propensity matched analysis except for coronary artery dissection (odds ratio [OR]: 0.10; 95% confidence interval [CI]: 0.02-0.65, p = 0.016) and ischemic stroke (OR: 0.35; 95% CI: 0.14-0.93, p = 0.034) which were lower in the CML group. CONCLUSION: This analysis showed no statistically significant difference in mortality, 30-day readmission, and post PCI complications rates between CML and non-CML patients. However, interestingly, CML patients may experience lower coronary artery dissection and ischemic stroke events than those without CML diagnosis.

The use of urodynamic to assess the mechanism of incontinence in patients with Yang-Monti based catheterizable cutaneous stomas.

OBJECTIVE: To analyze the static and dynamic urodynamic parameters of reservoirs and continent conduits in continent cutaneous urinary diversion with catheterizable stoma. MATERIALS AND METHODS: 76 patients had augmented ileocystoplasty or continent urinary diversion with catheterizable urinary stoma based on Mitrofanoff principle and Yang-Monti procedure using subserous tunnel as continence mechanism. They were followed up for at least 6 months post-operatively for continence through stoma and divided into two groups (continents vs non-continent) according to stomal continence. Both groups had urodynamic assessment performed via the stoma to assess reservoir capacity, pressure and contractions, efferent limb functional length, reservoir overactivity, static and dynamic maximal closure pressures and leak point pressure. RESULTS: Continence rate was 87%. Continent group included 66 patients and incontinent group included 10 patients. In both groups at rest, the reservoir pressure after filling did not exceed 25 cm H2O. During peristaltic contraction, the pressure did not exceed 30 cm H2O and the duct remained continent. After Valsalva maneuver, the reservoir pressure increased up to 34 (+ 7.4) cm H2O and leakage occur in 10 patients (13%). Reservoir (wall) overactivity was recorded in 54 patients, with insignificant rise in intraluminal pressure during the contractions. In both groups, the efferent tract closing pressure was always higher than the reservoir pressure. The mean of maximal closing pressure at Valsalva was 82.5 (+ 4.18) cm H2O in the continent group and 61.66 (+ 8.16) cm H2O in the incontinent group. The mean functional length of the conduit was 4.95 + 1.62 in the continent group and 2.80 + 1.50 cm in the incontinent group. CONCLUSIONS: Urodynamic evaluation of continent catheterizable cutaneous stoma after Yang-Monti procedure has a practical significance. Functional length of the conduit seems to be the most influential factor for continence reflecting static & dynamic maximal closure pressure. Higher conduit closing pressure is associated with better continence. Contractions of the pouch and peristaltic contraction of the conduit has no effect on continence mechanism.

Outcomes following medial open reduction in infants aged ≤6 months with developmental dysplasia of the hip.

Aim: To evaluate mid-long term radiological outcomes following early medial approach open reduction (MAOR) performed for developmental dysplasia of the hip (DDH) in infants aged ≤6 months old at time of surgery, specifically incidence of clinically significant avascular necrosis (AVN), Severin outcomes and rates of further surgery. Methods: This is a single centre retrospective study of patients treated from 1999 to 2017. Only infants aged ≤6 months old at time of MAOR, and aged at least 6 years old at latest follow-up were included (minimum 5.5 years follow-up). Data was collected from electronic healthcare records and serial radiographs reviewed to assess outcomes. AVN was classified according to Kalamchi and MacEwen, with types 2-4 considered clinically significant. Severin classes I (excellent) and II (good) were considered satisfactory outcomes, and classes III + considered unsatisfactory. Results: MAOR was performed on 48 hips in 44 patients. Mean age at time of surgery was 4 months (SD 1.4, range 2-6), with mean follow-up of 9.8 years (SD 2.7, range 6.2-16.2). Clinically significant AVN developed in 9/48 hips (19 %), all of which were type 2. Only 1/48 hips (2 %) required a subsequent pelvic osteotomy due to residual dysplasia with subluxation at 2 years post MAOR. At final follow-up, 81 % of patients had excellent or good radiological outcomes (Severin I/II). No statistically significant predictors for developing AVN, including age and presence of ossific nucleus, were identified. Conclusion: Early MAOR in infants aged ≤6 months was associated with a very low rate of significant residual dysplasia requiring further surgery, yet was not associated with unacceptable rates or severe forms of AVN. We therefore recommend MAOR is performed early to optimise acetabular remodelling potential and minimise the need for concurrent or subsequent bony procedures.

Cost-effectiveness of l-glutamine versus crizanlizumab for adults with sickle cell disease: model focused on reducing pain episode costs from Qatar's healthcare perspective.

Objectives: Treatment options for preventing vaso-occlusive crises among sickle cell disease patients are on the rise, especially if hydroxyurea treatment has failed. This economic analysis is conducted to assess the comparative clinical effectiveness, safety, and acquisition cost of l-glutamine and crizanlizumab for older adolescents and adults (⩾16 years old) with sickle cell disease in Qatar, with an emphasis on treatment costs and acute pain crises. Methods: We conduct a decision-tree model, where we compare the clinical and economic outcomes of two novel Food and drug administration (FDA)-approved medications which are available in Qatar; l-glutamine and crizanlizumab over a time horizon of 1 year in a hypothetical cohort of adult sickle cell disease patients from a Qatar healthcare perspective. The main outcome is incremental cost per sickle cell disease-related acute pain crises averted. Model clinical parameters were derived from individual drug randomized trials, published literature, whereas cost parameters from Qatar healthcare payer system (2020-2021). A sensitivity analysis was carried out, and the study results were robust around model inputs. Costs were converted to 2020 US dollars. Results: Study results showed that both treatment modalities' costs were the main driver of this analysis, with an average annual cost of the treatments per patient being $189,014 for crizanlizumab (5 mg/kg), $143,798 for crizanlizumab (2.5 mg/kg), and $74,323 for l-glutamine. The probability of no first-time sickle cell disease-related vaso-occlusive crises averted was 0.001/year for glutamine, 0.26/year for crizanlizumab (5 mg/kg), and 0.34/year for crizanlizumab (2.5 mg/kg). Lower dose crizanlizumab (2.5 mg/kg) dominated the higher one (5 mg/kg). The incremental cost-effectiveness ratio of crizanlizumab (2.5 mg/kg), when compared to l-glutamine was $81,265 per sickle cell disease-related vaso-occlusive crises averted. When comparing crizanlizumab (5 mg/kg) and l-glutamine, crizanlizumab (5 mg/kg) showed higher efficacy, yet the crizanlizumab incremental cost-effectiveness ratio was at $459,620 than l-glutamine. Conclusions: Crizanlizumab (2.5 mg/kg) may be a cost-effective intervention, yet it is not the approved dose for preventing vaso-occlusive crises in adolescents and adults with sickle cell disease. Crizanlizumab (5 mg/kg) was more cost-effective than the approved l-glutamine per sickle cell disease vaso-occlusive crisis prevented. Of note, we primarily focused on modeling acute vaso-occlusive pain, which limited our ability to consider other key outcomes in sickle cell disease.

Luspatercept's use in a patient with transfusion-dependent beta-thalassemia and intrathoracic extramedullary hematopoiesis (EMH).

Key Clinical Message: This case report and literature review examine the use of a relatively novel agent in a transfusion-dependent beta-thalassemia patient with extramedullary hematopoiesis (EMH). It examines the benefits and risks associated with its use and reviews the available literature while highlighting the drug's results in our patient with a higher risk profile. Abstract: Beta thalassemia can be complicated by EMH, which causes different symptoms based on location and size. Luspatercept is a new agent approved for transfusion-dependent thalassemia and Non-transfusion-dependent thalassemia (NTDT). Still, its use in patients with EMH was not well studied, and literature showed an increased risk of EMH expansion or development of new masses after its use. We discuss, in this case, the results of luspatercept treatment in a patient with transfusion-dependent thalassemia who is considered high risk for its use due to the patient's specific characteristics (history of symptomatic intrathoracic EMH, previous splenectomy, refusal to use antithrombotic medications). While also highlighting the benefits of using luspatercept regarding decreasing the iron overload and improving hemoglobin levels and examining how it was used safely to manage a transfusion-dependent thalassemia patient with an extramedullary hematopoiesis mass with no adverse events of note.

Potential efficacy of crizanlizumab in treating priapism in sickle cell disease: A case report.

This report documents the treatment of a 41-year-old male with sickle cell disease (SCD) and repeated stuttering priapism using crizanlizumab, which alleviated the priapism but induced a significant vaso-occlusive crisis during the second infusion. Encouragingly, no subsequent vaso-occlusive crises occurred. However, the potential for infusion-related adverse events warrants close supervision. Further research is necessary to explore its full benefits on priapism management.

The characteristics of CALR mutations in myeloproliferative neoplasms: a clinical experience from a tertiary care center in Qatar and a literature review.

BACKGROUND: Myeloproliferative neoplasms (MPNs) are hematological disorders characterized by abnormal production of myeloid cells due to genetic mutations. Since 2013, researchers have identified somatic mutations in the Calreticulin (CALR) gene, primarily insertions or deletions, in two Philadelphia chromosome-negative MPNs; essential thrombocytosis (ET) and primary myelofibrosis (PMF), and occasionally in chronic myelomonocytic leukemia (CMML). This study aims to identify the various types of CALR mutations and their impact on CALR-positive MPN patients' clinical manifestations and outcomes. METHODS: A single-center retrospective study was conducted. The data was collected from pre-existing records. The study was carried out on Philadelphia-negative MPN patients who were being followed up on at the NCCCR (National Center for Cancer Care and Research) to assess the clinical manifestation and outcome of disease treatment. All patients included, were followed in our center between January 1, 2008, and November 20, 2021. RESULTS: A total of 50 patients with CALR-positive MPN were reviewed with a median follow-up of three years (1-11). This cohort included 31 (62%) patients with ET, 10 (20%) patients with PMF, and 9 (18%) patients with prefibrotic myelofibrosis (pre-MF). The study involved 38 (76%) male and 12 (24%) female patients. There were 16 (32%) patients diagnosed before the age of 40, 24 (48%) patients diagnosed between the ages of 40 and 60; and 10 (20%) patients diagnosed after the age of 60. Molecular analysis showed 24 (48%) patients with CALR type 1, 21 (42%) patients with CALR type 2, and 5 (10%) patients with none Type 1, none Type 2 CALR mutations. Two patients have double mutations; 1(2%) with none Type 1, none Type 2 CALR and JAK2 mutations, and 1(2%) with CALR type 1 and MPL mutations. The thrombotic events were 3 (6%) venous thromboembolisms, 3 (6%) abdominal veins thromboses, 2 (4%) strokes, and 4 (8%) ischemic cardiac events. Only 4 (8%) patients progressed to Myelofibrosis and were carrying CALR 1 mutations, and 1 (2%) patient progressed to AML with CALR 2 mutation. CONCLUSION: The data shows a significant rise in CALR-positive MPN diagnoses in younger people, emphasizing the need for a better assessment tool to improve disease management and reduce complications.

Clinical and Environmental Surveillance for the Prevention of Legionellosis.

Legionella is a Gram-negative bacterium whose natural hosts are aquatic protozoa, in which the microorganism replicates and is protected from adverse environmental conditions [...].

Factors associated with usability of the EMPOWER-SUSTAIN Self-management mobile app© among individuals with cardiovascular risk factors in primary care.

Objective: This study aimed to determine the usability of the EMPOWER-SUSTAIN Self-Management Mobile App© and evaluate the factors associated with its usability among patients with cardiovascular risk factors in primary care. Methodology: This was a cross-sectional study, conducted among patients aged ≥ 18 years with cardiovascular risk factors attending a university primary care clinic. Patients were given the app to use for at least three months. Those who fulfilled the eligibility criteria were recruited. Data gathered were on sociodemographic, clinical characteristics, self-management support by doctors, utilisation of the app at home and social support in using the app. The previously translated and validated Malay version of the mHealth App Usability Questionnaire was used to measure usability. The mean usability score was calculated and linear regressions analysis was conducted to determine the factors associated with the usability of the app. Results: A total of 247 patients with at least one cardiovascular risk factor(s) were recruited. The mean age was 60.2 (±8.2). The majority were Malays (86.2%) and half of them were males (52.2%). The total mean (±SD) usability score was 5.26 (±0.67) indicating a high usability of the app. Usability of the app declined with increasing age in the simple linear regressions analysis. The multiple linear regressions yielded that being Malay (b = 0.31, 95% CI 0.08,0.54), using the app at home to understand their medications (b = 0.33, 95% CI 0.12,0.53) and having social support from family members and friends (b = 0.28, 95% CI 0.07,0.49) were significantly associated with higher usability of the app. Conclusion: The usability of the EMPOWER-SUSTAIN Self-Management Mobile App© was high among patients with cardiovascular risk factors in our primary care clinic. This finding supports the widespread use of this app among our patients. Involvement of family members and friends should be encouraged to improve the usability of the app.

A practical guide to the management of immune thrombocytopenia co-existing with acute coronary syndrome.

Introduction: Immune thrombocytopenia (ITP) management with co-existing acute coronary syndrome (ACS) remains challenging as it requires a clinically relevant balance between the risk and outcomes of thrombosis and the risk of bleeding. However, the literature evaluating the treatment approaches in this high-risk population is scarce. Methods and Results: In this review, we aimed to summarize the available literature on the safety of ITP first- and second-line therapies to provide a practical guide on the management of ITP co-existing with ACS. We recommend holding antithrombotic therapy, including antiplatelet agents and anticoagulation, in severe thrombocytopenia with a platelet count < 30 × 109/L and using a single antiplatelet agent when the platelet count falls between 30 and 50 × 109/L. We provide a stepwise approach according to platelet count and response to initial therapy, starting with corticosteroids, with or without intravenous immunoglobulin (IVIG) with a dose limit of 35 g, followed by thrombopoietin receptor agonists (TPO-RAs) to a target platelet count of 200 × 109/L and then rituximab. Conclusion: Our review may serve as a practical guide for clinicians in the management of ITP co-existing with ACS.