Sickle cell disease (SCD) is marked by episodic vaso-occlusive crisis (VOC). Recurrent VOC creates a pro-inflammatory state that induces phenotypic alterations in innate immune cells. Monocytes are of particular interest to VOC pathophysiology because they are especially malleable to inflammatory signaling. Indeed, inflammatory disease states such as chronic obstructive pulmonary disease (COPD), obesity and atherosclerosis are known to influence monocyte development and alter monocyte subpopulations. In this study, we describe SCD monocyte subsets by performing immunophenotypic flow cytometric, enzymatic, and morphologic analysis on peripheral blood. Herein, we add to the growing body of evidence suggesting aberrant monocyte populations underpin VOC pathophysiology. We found that SCD monocytes possess an immature phenotype as demonstrated by 1) decreased CD4 positivity (p < .01), 2) low α-naphthyl butyrate esterase (ANBE) expression, and 3) naïve morphologic features. We additionally found an increase in CD14+CD16-CD4- monocytes (p < .01), a subset associated with the impaired immune response of post-trauma patients. Interestingly, we also found a large proportion of CD14+CD4-HLA-DR- monocytes which, under normal circumstances, are exclusively found in neonates (p < .01). Finally, we report an increase in nonclassical monocytes (CD14dimCD16+), a subset recently shown to have a critical role in prevention and recovery from VOC.

This report of two cases confronts the longstanding perception of Sickle Cell Trait (SCT) as a clinically benign condition, highlighting its complex and severe clinical manifestations, particularly in the context of blood loss anemia and vaso-occlusive crises (VOCs). The hallmark of sickle cell disease is the severe pain caused by acute vaso-occlusion of the microvasculature that leads to bone marrow infarction. We report two cases of patients with SCT and severe anemia in the setting of blood loss secondary to uterine fibroids subsequently causing VOCs with likely bone sequestration. The occurrence of VOCs in SCT, while infrequent, can be serious and demands a high index of suspicion, particularly when patients appear in significant distress and cardiac or vascular etiologies are ruled out as a source. Reversal of anemia in this case provided quick resolution to symptoms, and we recommend other clinicians not disregard a differential of VOC in SCT carriers, and urge to treat patients as they would if they had sickle cell disease. This report challenges the conventional view of SCT as a condition of clinical benignity, calling for a recalibration in the clinical understanding, management strategies, and focus on this genetic trait under similar circumstances.

Cholera meningitis is a rare complication of Vibrio cholerae (V. cholerae) infection. We present a case of cholera meningitis caused by toxigenic V. cholerae O1 in a 34-year-old male with sickle cell disease (SCD). The patient presented with fever, diarrhea, and altered mental status. Cerebrospinal fluid (CSF) analysis showed 5,231 cells/μL (53.9% neutrophils), a protein level of 462 mg/dL, and a glycorrhachia level of 26 mg/dL. V. cholerae O1 was isolated on CSF culture. Despite the patient undergoing antimicrobial therapy, brain imaging revealed basal ganglia ring-enhancing lesions suggestive of tuberculomas. Antituberculosis treatment and steroids led to clinical improvement. This report highlights the need to consider V. cholerae meningitis in patients with SCD who present with diarrhea and altered mental status. Prompt diagnosis and appropriate antimicrobial therapy are keys to improving patient outcomes.

Sickle cell disease (SCD) is a chronic and prevalent hemoglobin disorder with various manifestations and complications depending on the organs involved. Red cell transfusion either simple or exchange is crucial due to its prophylactic and therapeutic roles. We present a case showing serologic discrepancy between the red cells phenotype and the developed alloantibodies to emphasize the crucial role of molecular testing in SCD patients requiring chronic blood transfusion.

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has been widely documented as a multi-systemic illness and associated with an increased incidence of thromboses. Likewise, sickle cell disease (SCD) is a hematologic disease responsible for widespread effects on the vasculature and is also associated with elevated thrombotic risk. In this review, we examine the incidence rates of venous thromboembolism (VTE) in SCD and COVID-19 independently and review the mechanisms of coagulopathy associated with both diseases. We describe the possible associations and commonalities between VTE mechanisms, as both diseases cause widespread inflammation that influences each tenet of Virchow\'s triad. We also discuss current anticoagulation guideline recommendations for the prevention of VTE events in each of these diseases. We report on current literature to date describing rates of VTE in SCD-COVID-19 patients and outline prospective areas of research to further understand the possible synergistic influence of coagulopathy in these patients. The association between SCD and COVID-19 remains a largely under-researched area of coagulopathy in current hematology and thrombotic literature, and our report lays out potential future prospects in the field.

The association between Moyamoya syndrome (MMS) and sickle cell disease (SCD) has been well-established in pediatric populations; however, limited literature exists documenting the characteristics and management of MMS in adult SCD patients. Studies have indicated the role of endovascular management in secondary stroke prevention for pediatric populations, with no current guidelines available for adult populations. Here, we describe a unique case of MMS in a 30-year-old patient with SCD and incidental protein S deficiency. Our unique case highlights a patient at high risk for neurosurgical intervention due to her hypercoagulable state who has benefitted from medical management. We also discuss current literature for the prevention of secondary cerebral vascular events and the role of further studies involving adult populations with MMS and SCD.

Background The study was conducted to find the prevalence of hemoglobinopathies along with their geographical/ethnic distribution to highlight the region of high prevalence that can be used to guide screening. Method Results of blood samples received for hemoglobin variants determination by high-performance liquid chromatography (HPLC) were retrospectively analyzed at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore. Blood samples were assayed for CBC (complete blood count), red blood cell morphology, and hemoglobin analysis by HPLC. CBC was performed on Sysmex XN 9000 analyzer (Sysmex, Kobe Japan), peripheral smears to review RBC morphology were stained with Wright-Giemsa stain, and HPLC was performed on BIO-RAD variant II (Bio-Rad Laboratories, Hercules, USA). Results Hemoglobinopathies were identified in 9.7% (n=997) out of 10,297 samples. Beta thalassemia trait was the most common hemoglobinopathy recognized with a prevalence of 5% (n=516), with the maximum number of cases in the Lahore district of Punjab province. The next most common hemoglobinopathy identified was sickle cell disease with a frequency of 1.43% (n=148) and the maximum cases from the Dera Ismail Khan district of the Khyber Pakhtunkhwa province. The additional important hemoglobinopathies found were sickle cell trait, hemoglobin-D Punjab trait, and compound heterozygote for sickle and beta thalassemia. Conclusion Hemoglobinopathies are the most common inherited disorders in Pakistan and worldwide. Screening for hemoglobinopathies is recommended in high-prevalence districts of Pakistan. Sickle cell screening is also recommended in newborns in the high prevalence area of Pakistan, such as the northwest regions.

Sickle cell disease (SCD) is a hematological disorder that is inherited in an autosomal recessive (AR) fashion. It is caused by mutations in the genes encoding for the globin apoprotein of hemoglobin (Hb), leading to diminished oxygen-carrying ability. Its pathophysiologic mechanism affects multiple organ systems, making it crucial to understand the complications of SCD and find the best ways to prevent and treat them. Some important ways that SCD manifests in the respiratory system are acute chest syndrome (ACS), pulmonary hypertension (PH), asthma, and venous thromboembolism (VTE). This article summarizes their salient features, including pathogenesis related to the adverse outcomes, screening practices, and management guidelines, with the intent to provide greater insight into forming better practices that increase the quality of life in SCD patients.

Sickle Cell Disease (SCD) is a disease that affects many around the world and often accounts for frequent hospital admissions every year, secondary to uncontrolled pain. Marijuana is increasingly being used for its medicinal ability to treat pain in chronic medical conditions. Therefore, it is imperative to determine how effective it would be in providing pain relief to patients with SCD. We systematically screened five databases for relevant data: PubMed, Medline, PubMed Central (PMC), Cochrane Library, and Google Scholar. The inclusion and exclusion criteria were implemented. A quality appraisal was then done using the Cochrane Bias assessment for randomized controlled trials (RCTs), Newcastle-Ottawa tool for observational studies, and Scale for the Assessment of Narrative Review Articles (SANRA) checklist for traditional review articles. From seven articles, information was gathered; one systematic review, one RCT, two surveys, one cross-sectional study, one retrospective study, and one questionnaire-based study. Our review concluded that based on the literature assessed, marijuana use in SCD patients either worsened their painful crises or offered little to no help compared to opioids or hydroxyurea usage. There were limited RCTs published in addition to papers investigating the long-term effects of marijuana use in SCD. We hope that further data is gathered in these areas to sufficiently address whether cannabis use is efficacious for pain relief in patients with SCD.

Sickle cell disease variants can commonly present as life-threatening complications, like sequestration crisis, hypersplenism, or stroke. However, clinicians should also look for milder findings like asymptomatic chronic anemia mimicking iron deficiency as a milder, more insidious clue to an underlying sickle cell variant. Early investigations of these milder symptoms can potentially reduce the risk of more severe complications such as vaso occlusive crisis. In this report, we present a 75-year-old African-American female, who was referred to the hematology clinic for chronic anemia without any history of vaso occlusive crisis and was eventually diagnosed with sickle cell beta plus thalassemia as per hemoglobin electrophoresis. Here, we review the challenges in diagnosing rarer types of sickle cell disease and the importance of educating patients about the diagnosis. This rare type demands clinicians\' awareness to identify the disease early and to understand the etiology of the complications, if any, that occur.