This systematic review aims to determine the incidence and prevalence of acute stroke in patients with sickle cell disease (SCD) and explore its clinical implications and outcomes. A comprehensive search was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines to identify relevant studies published between 2013 and 2023. Databases including PubMed, Google Scholar, ResearchGate, ScienceDirect, and Wiley Online Library are searched. Inclusion criteria are applied to select studies involving human subjects, written in English, and reporting the association between acute stroke and confirmed cases of SCD. Study quality assessment and data extraction are performed by two independent reviewers using study-specific tools. A total of 9 studies met the inclusion criteria including 7 cohort studies, and two meta-analyses are included in the systematic review with a total of 24,480 participants. The prevalence of stroke in SCD patients varied across studies, ranging from 1.24% to 27.3%. The incidence rates of stroke showed significant variability, ranging from 0.31% to 31%. Long-term disabilities, recurrence, and reduced quality of life characterized the clinical outcomes of stroke in SCD patients. Mortality rates varied among studies, emphasizing the need for early detection and comprehensive care. The review provides valuable insights for health-care professionals, researchers, and policymakers involved in SCD care, emphasizing the importance of early detection, intervention, and comprehensive management to improve patient outcomes. Further research is needed to address knowledge gaps and understand the underlying mechanisms of stroke in SCD.

INTRODUCTION: In India, there is a high burden of infections such as tuberculosis, dengue, and malaria which are common causes of monocytosis. This increases the workload of smear examination by the pathologist. “Mono-dysplasia score” is obtained with a simple complete blood count on an automated cell counter and includes the parameters neutrophil (Ne) and monocyte (Mo) count and Ne-WX which is a Ne dispersion parameter. It is operator independent, objective, and does not require a high level of expertise. AIMS: The aims of the study were to assess the utility of Monoscore/mono-dysplasia score calculated using research parameters of Sysmex XN automated cell counter, as a screening tool for differentiating reactive monocytosis from hematological malignancies associated with monocytosis. MATERIALS AND METHODS: Samples sent in EDTA vacutainer for routine hemogram fulling the criteria for monocytosis (WHO criteria – absolute monocyte count ≥1 × 10⁹/L and accounting for ≥10% of the total white blood cell count) were included in the study. Monoscore was calculated using the formula established by Schillinger et al. Flow cytometry, bone marrow examination, etc., were done as and when needed as standard-of-care tests to establish a final diagnosis. RESULTS: One thousand two hundred and fifty-seven samples were analyzed out of which 41 samples were chronic myelomonocytic leukemia and 126 were other hematological malignancies (HD) including acute leukemias, myelodysplastic syndrome, myeloproliferative neoplasm, etc. Using receiver operating characteristics curves, we established the cutoff 0.212 which showed a sensitivity of 97.6% and specificity of 96.4% to differentiated reactive monocytosis form HD. CONCLUSIONS: A sample showing monocytosis and Monoscore <0.212 and without any other flags can be safely auto-authorized without peripheral blood smears examination, reducing the burden of slides to be reviewed.

BACKGROUND: Lymphoma is a cancer of the lymphatic system with two main types, namely Hodgkin lymphoma (HL) and non-HL (NHL). Within these, there are many subtypes. Based on data from the National Cancer Institute, NHL accounts for 4.2% whereas HL accounts for 0.5% of all cancers in the United States. The treatment strategy depends on the type and the stage of the lymphoma. In this study, we are studying the incidence rate of lymphoma in a military-nononcology center for better understanding and recording of the condition. METHODS: We conducted an observational, analytical, retrospective study in a tertiary care hospital on 56 patients admitted to the hospital between January 2016 and the end of December 2021, with lymphadenopathy or body mass, who were diagnosed with lymphoma. The modality of diagnosis was tissue histopathology. All data including demographics, details of lymphoma lesions, and laboratory and histopathology results were obtained from health informatics or manual records. RESULTS: In this study, we found that 67.9% of the cases were male, with a significantly higher rate among Saudi patients in comparison to non-Saudi (94.6% and 5.3%, respectively). Among Saudi cases, we found that a significantly higher percentage of lymphoma cases were originally from the Eastern and the Southern provinces (47.1% and 35.8%, respectively), and 46.5% of them were living in Dammam at the time of diagnosis. The percentage of NHL was significantly higher than Hodgkin type (80.3% and 19.6%, respectively). The majority of the cases belonged to B-cell NHL (73.2%). Moreover, the incidence rate increased from 2016 to 2021 with an accumulative rate of 25.14 / 100,000 person-years. CONCLUSION: Our data indicate that the incidence rate of lymphoma increased in the last 5 years at a tertiary care center. We found evidence for associations between the original province and the rate of the lymphoma. Moreover, the rate of B-cell NHL was significantly higher with superior mortality rate in comparison to other types of lymphoma in our cohort, which supported previous studies in the Kingdom of Saudi Arabia. There was no increase in incidence of lymphoma in our study cohort of military personnel, and it matches the incidence data of other local studies. Further national studies and initiation of lymphoma registry may add a value for future researches.

BACKGROUND: For patients affected by haemophilia, Patient Support Programs (PSPs) may be a support tool to assist patients and physicians in optimising treatment and improving disease management. PSP on the Pharmacokinetics (PK) service in Italy aims to support haemophilia A or B patients. MATERIALS AND METHODS: This report analyses data of patients and physicians who benefited from the PK program from October 2018 to October 2022. Data was collected by a web-based questionnaire and by telephone calls. We provide a descriptive report on the benefits of participation in the PK program for patients with haemophilia and medical professionals involved in their care. RESULTS: In total, 69.6% of the respondent patients stated they were very satisfied with the Program, and 97.7% said they would benefit from the service again. Among the interviewed physicians, 60% felt satisfied with the Program and thought it was helpful. CONCLUSION: Patient satisfaction remained consistently high, with sustained improvements in all aspects of life. Medical professionals also reported positive outcomes in their patient interactions and satisfaction with the Program. PSPs have empowered patients to manage their pathology, increasing their satisfaction with treatments and quality of life.

BACKGROUND: Beta-thalassemia is an autosomal recessive hereditary blood disorder occurs due to absent or diminished β-globin chains synthesis. The transformation from fetal to adult in globin synthesis is controlled by different mechanisms; one of those is BCL11A which is downregulated by microRNA-210 (miRNA-210). Therefore, this would lead to reactivation of fetal hemoglobin (HbF) production that improves anemia and reduces the needs for transfusion. OBJECTIVE: To study miRNA-210 in the plasma of beta thalassemia patients and its relation to HbF level and disease severity. SUBJECTS AND METHODS: The study was carried out on 50 individuals divided into three groups. Group A included 30 thalassemia major patients, Group B included 10 thalassemia intermedia patients, and Group C is 10 healthy volunteers as control. All individuals underwent to history taking, physical examination, and laboratory investigations, complete blood count, serum ferritin, hemoglobin electrophoresis, and measurement of miRNA-210 expression levels in plasma of peripheral blood samples by quantitative real-time polymerase chain reaction. RESULTS: Examination of miRNA-210 plasma level among different groups revealed no statistically significant association between control and thalassemia group or its subgroups (Major and intermedia) (P = 0.224, P = 0.116, and P = 0.734, respectively). Moreover, miRNA-210 was slightly higher in responders than nonresponders to hydroxyurea but did not reach significant level. CONCLUSION: miRNA-210 levels were not associated with increase in HbF levels in thalassemia patients.

BACKGROUND: Coronavirus disease 2019 (COVID-19) globally known as COVID-19 is a heterogeneous disease caused by the severe acute respiratory syndrome coronavirus 2. COVID-19 patients having severe disease and are critically ill are more prone to have abnormal coagulation prolife. It may lead to thromboinflammation in severe conditions. AIM: The aim is to evaluate the utility of coagulation parameters and inflammatory biomarkers in COVID-19 patients in the study conducted for 6 months duration. MATERIALS AND METHODS: It was a retrospective observational study conducted from April 2021 to September 2021 in a rural tertiary care referral Institute. Available data of the baseline values of coagulation parameters (platelet count [PLC], prothrombin time [PT], international normalized ratio [INR], partial thromboplastin time [PTT] and D-dimer) and inflammatory markers (C-reactive protein [CRP], serum lactate dehydrogenase [LDH], and serum ferritin) were evaluated with respect to the clinical severity of the disease and also the survival status of COVID-19 patients. RESULTS: One hundred and fifty-five cases of COVID-19-positive patients were analyzed. PT, INR, PTT, D-dimer, CRP, LDH, and ferritin had higher mean in severe cases than the nonsevere cases. PLC had higher mean in the severe cases than the nonsevere cases. PT, INR, PTT, D-dimer, CRP, LDH and ferritin had higher mean in nonsurvivors than the survivors. PLC had lower mean in nonsurvivors than the survivors. CONCLUSION: Baseline values of PT, INR, PTT, D-dimer, LDH, and ferritin may be considered reliable indicators of severity of COVID-19 disease. Baseline values of D-dimer, LDH, and ferritin may be considered a reliable indicator of survival of patients suffering from COVID-19 infection.

BACKGROUND: Neonatal sepsis is a major health problem in our country. The cause for most of the deaths is not a failure to treat the infection, instead, it is the failure to treat it on time. Sepsis is usually identified by a gold-standard blood culture test. However, there is a need for a simple and cost-effective diagnostic tool with quick results. MATERIALS AND METHODS: A total of 70 neonates with clinical suspicion of sepsis were included in our study. The hematological parameters such as total leukocyte count(TLC), absolute neutrophil count (ANC), platelet count, immature neutrophil count, immature to total neutrophil ratio (I:T), immature to mature neutrophil ratio (I:M), toxic granules, and cytoplasmic vacuolation in neutrophil were correlated with C-reactive protein (CRP), blood culture and micro-erythrocyte sedimentation rate (micro-ESR). The performance of these hematological parameters was evaluated with the help of hematological scoring system (HSS). RESULTS: Among the hematological parameters, TLC has high sensitivity (77.8%) and specificity (72.1%), ANC has the least sensitivity (44.4%) but the highest specificity (96.7%), platelet count has high sensitivity (88.9%) but low specificity (44.3%), I:T and I:M ratio have the highest sensitivity (88.9% both), specificity (71.1% and 88.5%, respectively) and negative predictive value (91.3% and 92.8% respectively). ANC and I:M ratios have the highest positive predictive value (89.3% and 82.5%, respectively) in predicting the early diagnosis of neonatal sepsis. CONCLUSION: HSS along with CRP and micro-ESR can be used as a screening tool in neonates with clinical suspicion of sepsis as they are simple to perform, and can be done even in rural parts of the country.

BACKGROUND: Thromboembolic (TE) disorders are among the most common complications of beta-thalassemia. We designed this cross-sectional study to investigate the state of hypercoagulability and platelet activation in patients with beta-thalassemia. PATIENTS AND METHODS: Seventy-five patients diagnosed with beta-thalassemia by hemoglobin electrophoresis were divided into three groups of 25 patients each: Group I (thalassemia trait), Group II (thalassemia intermedia [TI]), and Group III (thalassemia major). In addition, 50 healthy individuals were included as controls. Both patients and control groups were subjected to clinical and laboratory assessment, which included measurement of protein C, anti-thrombin III, D-dimer, CD41, CD42, CD61, and CD62P, and detection of beta-chain mutations. RESULTS: Levels of the platelet activation marker CD62P were significantly higher in beta-thalassemia patients (26.28 ± 18.01%) than in controls (4.78 ± 2.27%) (P < 0.001). The D-dimer level was significantly higher in beta-thalassemia patients (348.41 ± 571.01 ng/mL) than in controls (71.6 ± 39.61 ng/mL) (P < 0.001). Protein-C and AT-III levels were significantly lower in beta-thalassemia patients (71.45 ± 13.26%, 78.38 ± 15.32%) in comparison with controls (94.9 ± 13.03%, 96.52 ± 11.01%) (P < 0.001 and P < 0.001, respectively). TE disorders were found in 7/25 (9%) beta-thalassemia patients, especially in older and postsplenectomy patients. TE was most commonly found in beta-TI. Beta-chain mutations were found in all patients with TE disorders, especially compound heterozygous intervening sequence (IVS) (IVS1.6 [T > C]/IVS1.110 [G > A]). CONCLUSION: Postsplenectomy teenagers and adults with beta-thalassemia with lower levels of natural anticoagulant in the blood, an increased level of D-dimer, and platelet activator factor had a significantly higher risk for TE than those with childhood beta-thalassemia and the control group. In comparison with other beta-thalassemia patients, TI with beta-chain mutations is more likely to develop TE.

BACKGROUND: Despite optimal transfusion and chelation therapy, beta-thalassemia patients still commonly experience various complications, including renal complications, which may affect their survival. This case–control study involved pediatric patients with beta-thalassemia major (β-TM). MATERIALS AND METHODS: This case–control study involved pediatric patients with β-TM, a total of 60 patient samples were randomly collected from the Genetic Hematology Center at the Dhi-Qar Health Directorate, with an age range of 1–14 years. In addition, 60 samples were collected from healthy children in the same age range as the control group. In this study, the glomerular injury was predicted by measuring the concentration of serum cystatin-C (Cys-C), and the tubular injury was predicted by measuring the concentration of serum netrin-1 (NTN-1). Routine markers, such as hemoglobin (Hb) level, ferritin, serum albumin, and creatinine, were also examined for the patients and the control group. RESULTS: The results showed a statistically significant increase in serum Cys-C in the patient group compared to the control group (P < 0.001) and a significant increase of serum NTN-1 in β-TM patients compared to the control group (P < 0.001). In addition, the study showed a statistically significant decrease in Hb, serum albumin, and creatinine and a significant increase in serum ferritin in the patients group compared to the control group. In conclusion, from the results of this study, we can conclude that the serum Cys-C and NTN-1 measurements can be used as predictive biomarkers for glomerular and tubular injury and increase with age. CONCLUSION: Predicting glomerular and tubular injury using the measurement of serum Cys-C and NTN-1 has improved significantly and advanced with age.

BACKGROUND: Sickle cell disease (SCD) is a genetic disorder characterized by hemolytic anemia and vaso-occlusive episodes. Many adults with SCD require hospitalization for the management of acute manifestations. There appears to be a gap in the level of knowledge that general internal medicine (GIM) trainees possess regarding the treatment and management of SCD. METHODS: We created an online module that helps medical residents improve their knowledge about SCD. Participants were exposed to different scenarios related to SCD. A pre-and posttests were administered to detect improvement in knowledge and comfort when managing SCD and its acute complications. RESULTS: Thirty consecutive GIM residents participated. Of these, 20 completed the demographic survey, 17 completed the pretest, and 10 completed the posttest and usability survey. The median time to complete the module was 68 min. Fifty percent of participants were 1^st-year postgraduation from medical school. Forty-five percent stated that they were uncomfortable managing patients with SCD 80% had cared for at least 1–5 patients with SCD. All reported having no SCD lectures in residency. The median pretest score was 12/20 (range, 8–15) and posttest score was 20.5/25 (range, 13–24). CONCLUSIONS: Residents agreed that the module was useful, helps understanding about SCD and its complication, and aids with clinical duties. This module demonstrated to be an effective educational tool that can support resident education during their internal medicine. Further strategies will be needed to improve the delivery of online modules to keep residents engaged with the module and attaint full benefit from these activities.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired rare disease that affects both males and females characterized by the triad of intravascular hemolytic anemia, thrombosis, and bone marrow failure. Flow cytometry is the gold standard for the final diagnosis. This is a PNH case of a 42-year-old Indonesian woman who presented to the emergency department with sustained generalized fatigability, pallor, and jaundice with a history of multiple recurrent previous events managed by blood transfusions. The diagnosis was reached by laboratory studies that revealed hemolytic anemia and erythroid hyperplasia, which was confirmed by a clonal deficiency of CD59 red blood cells (RBCs) using flow cytometry. She was prescribed eculizumab. Hemolytic anemia and jaundice in patients with PNH can be caused by RBCs hemolysis due to the absence of CD55 and CD59 proteins caused by a genetic defect (PIG-A) on the X chromosome. That is why flow cytometry must be made for each patient suspected of having PNH.

Anaplastic lymphoma kinase positive (ALK+ve) large B-cell lymphoma (LBCL) is a rare, aggressive subtype of diffuse LBCL with unique morphological characteristics and unusual immunophenotypic features that have substantial overlap with other hematologic and nonhematologic neoplasms. Here, we present a 48-year-old male who presented with progressive swelling on both sides of the neck and left eye followed by protruding mass in the left nose over a span of 6 months. Based on the biopsy findings of the left nasal mass, a plasma cell neoplasm was initially diagnosed; later on the diagnosis was modified as ALK + ve LBCL based on the aggressive clinical course and biopsy findings of cervical lymph node. The patient was started on chemotherapy and is tolerating well.