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Sickle cell disease (SCD) is the most common genetic blood disorder, characterized by red cell hemolysis, anemia, and corresponding increased compensatory cerebral blood flow (CBF). SCD patients are at high risk for cerebral infarcts and CBF quantification is likely critical to assess infarct risk. Infarcts primarily localize to white matter (WM), yet arterial spin labeling (ASL) MRI, the most common non-invasive CBF approach, has poor WM CBF sensitivity owing to low WM CBF and long WM bolus arrival time (BAT). We hypothesize that anemia, and associated cerebral hyperemia, in SCD leads to improved WM detection with ASL. We performed 3-Tesla multi-delay pulsed ASL in SCD (n = 35; age = 30.5 ± 8.3 years) and control (n = 15; age = 28.7 ± 4.5 years) participants and applied t-tests at each inversion time within different flow territories, and determined which regions were significantly above noise floor (criteria: one-sided p < 0.05). Total WM CBF-weighted signal was primarily detectable outside of borderzone regions in SCD (CBF = 17.7 [range = 12.9-25.0] mL/100 g/min), but was largely unphysiological in control (CBF = 8.1 [range = 7.6-9.9)] mL/100 g/min) participants. WM BAT was reduced in SCD versus control participants (ΔBAT = 37 [range = 46-70] ms) and BAT directly correlated with hematocrit (Spearman's-ρ = 0.62; p < 0.001). Findings support the feasibility of WM CBF quantification using ASL in SCD participants for appropriately parameterized protocols.

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Many adults with sickle cell disease experience chronic, non-vaso-occlusive pain that can benefit from non-pharmacological interventions available for use in the home setting. Virtual reality has been shown to be effective in decreasing pain in chronic pain conditions and may be useful for home-based self-management of chronic pain in sickle cell. However, the literature lacks studies examining this potential. Additionally, the knowledge and experiences of adults with sickle cell who have tried virtual reality for home-based chronic pain management have not yet been reported. This qualitative, descriptive pilot study explored the knowledge and perceptions of virtual reality among adults with sickle cell and their experience with using in-home virtual reality for chronic pain. Nine participants completed demographic questionnaires and an individual interview that was recorded, transcribed verbatim, and analyzed using thematic analysis. Participants were 21 to 38 years of age, and most were female (88.9%) with a medium or high sickle cell disease severity (88.9%) and a chronic pain grade classification of Grade III (high disability-moderately limiting) or Grade IV (high disability-severely limiting) (55.5%). Interview themes, which aligned with the Technology Acceptance Model, were: (1) pain beliefs and self-management; (2) virtual reality as another world; and (3) experience of using in-home virtual reality. Based on preliminary data, virtual reality shows promise as a strategy for non-pharmacological management of chronic pain in adults with sickle cell. However, further investigations are warranted to mitigate the challenges and limitations associated with using virtual reality in this capacity. PERSPECTIVE: Few evidence-based, non-pharmacological interventions exist for chronic pain in adults with sickle cell disease (SCD). This first qualitative, pilot study of in-home VR for chronic pain in adults with SCD suggests that VR interventions need further exploration as a non-pharmacological strategy for mitigating their pain in the home setting.

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Objectives: This study aimed to determine the health-related quality of life (HRQoL) of adult Omani patients with sickle cell disease (SCD). The quality of life of these patients in Oman is unknown. Methods: This cross-sectional study was conducted at the Sultan Qaboos University Hospital, Muscat, Oman, from June to October 2022 and included patients with SCD. A validated Arabic version of the 36-Item Short-Form Health Survey (SF-36) was used to assess HRQoL in 8 domains. Results: A total of 235 patients with SCD were included in this study, the majority of whom were female (74.9%) and between 18 and 35 years of age (64.6%). The lowest HRQoL was reported for the domain of role limitations due to physical health (median score = 25.0, interquartile range [IQR] = 35.0), while the highest was seen for role limitations due to emotional problems (median score = 66.7, IQR = 100.0). Frequent SCD-related emergency department visits/hospital admissions and the adverse effect of SCD on patients' social lives had a significant negative impact on SF-36 scores for all 8 HRQoL domains (P ≤0.05). Additionally, SCD's impact on academic performance and a history of having been bullied due to SCD had a significant negative impact on SF-36 scores for 7 domains (P ≤0.05). Conclusion: Omani adult patients with SCD reported relatively poor HRQoL in several domains, with various factors found to be significantly associated with this. Healthcare providers and policy makers should consider offering additional clinical, educational and financial support to these patients to enhance their HRQoL.

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Sickle cell disease (SCD), the most common autosomal recessive genetic disorder, affects the hemoglobin (Hb) chains in human red blood cells. It is caused by mutations in the ß-globin genes, leading to the production of hemoglobin S, which results in the formation of sickle-shaped red blood cells (RBCs). These abnormal cells cause hemolysis, endothelial damage, and small vessel occlusion, leading to both acute and long-term complications. According to the World Health Organization's 2008 estimates, SCD affects approximately 2.28 per 1000 individuals globally. Despite this high prevalence, therapeutic advancements have been slow. For many years, the only FDA-approved medications for managing SCD complications were hydroxyurea and deferiprone. However, recent years have seen the approval of several new therapies, including L-glutamine (2017), voxelotor and crizanlizumab (2019), as well as exagamglogene autotemcel (Casgevy) and lovotibeglogene autotemcel (Lyfgenia) (2023). These treatments have proven effective in managing both the acute and chronic effects of SCD, including hemolytic anemia, chronic pain, stroke, vaso-occlusive crises, and multiple organ damage syndromes. This review explores the mechanisms of action, practical considerations, and side effects of these emerging therapies, drawing from a comprehensive search of databases such as PubMed, Medline, and Cochrane.

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Ketamine is an N-methyl D-aspartate (NMDA) receptor antagonist used to treat pain at subanesthetic doses. Ketamine is beneficial for pain control in patients who have a high tolerance to opioids and are experiencing opioid-induced hyperalgesia. This study characterizes oral ketamine use for analgesia at a large academic hospital and reports safety outcomes for hospitalized patients. This study was a retrospective electronic health record (EHR) review of patients ≥ 18 years or older receiving oral ketamine. The primary endpoint was median ketamine starting dose and maximum dose (mg/kg/day) during treatment duration. Secondary outcomes included oral Morphine Milligram Equivalents (MMEs), buprenorphine dose, minimum and maximum pain scores on the first and last day of therapy. Safety endpoints were reported. The median starting dose was 1 mg/kg/day, and the median maximum dose was 1.6 mg/kg/day. Median MMEs decreased from the first day to the last day of oral ketamine therapy. The study population experienced a low incidence of safety events overall. Oral ketamine was administered safely for analgesia, with patients receiving ketamine doses that were on the lower end of the established therapeutic range. Evaluation of the efficacy and safety of oral ketamine use for analgesia should be further studied.

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BACKGROUND: Sickle cell disease (SCD) is a Mendelian disorder characterized by a point mutation in the ß-globin gene that leads to sickling of erythrocytes. Several studies have shown that absolute neutrophil count is strongly associated with clinical severity of SCD, suggesting an apparent role of white blood cells (WBC) in SCD pathology. However, the mechanism by which genetic variants lead to WBC count differences in SCD patients remains unclear. METHODS: Genome-wide association (GWA) analyses were carried out amongst a cohort of 2409 Brazil SCD participants. Association of WBC count and genetic markers were investigated in homozygous sickle cell anaemia participants and compound heterozygous sickle cell haemoglobin C participants. RESULTS: GWA analysis showed that variants in genes TERT, ACKR1, and FAM3C are associated with WBC count variation. The well-studied association between WBC count and Duffy null phenotype (variant in ACKR1) in healthy populations was replicated, reinforcing the influence of the SNP rs2814778 (T>C) in WBC count. CONCLUSION: Genetics plays an important role in regulating WBC count in patients with SCD. Our results point to possible mechanisms involved in WBC count variation and as increased WBC count is associated with more severe SCD, these results could suggest potential therapeutic targets for individuals with SCD.

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Sickle cell disease (SCD) is a major public health concern with significant associated economic costs. Although the disease affects all ethnic groups, about 90% of individuals living with sickle cell disease in the USA are Black/African American. The purpose of this study was to assess the health care discrimination experiences of adults living with SCD and the quality of the relationship with their health care providers. We conducted six focus groups from October 2018 to March 2019 with individuals receiving care at a specialized adult sickle cell program outpatient clinic at a private, nonprofit tertiary medical center and teaching hospital in the northeastern USA. The sample of 18 participants consisted of groups divided by gender and current use, past use, or never having taken hydroxyurea. Ten (56%) participants were males; most were Black/African American (83%) and had an average age of 39.4 years. This study reports a qualitative, thematic analysis of two of 14 areas assessed by a larger study: experiences of discrimination and relationships with providers. Participants described experiences of bias related to their diagnosis of SCD as well as their race, and often felt stereotyped as "drug-seeking." They also identified lack of understanding about SCD and poor communication as problematic and leading to delays in care. Finally, participants provided recommendations on how to address issues of discrimination.

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OBJECTIVE: This study characterized caregivers' beliefs related to early intervention services for children with sickle cell disease (SCD) to gain an indepth understanding of caregivers' experiences and desires for early intervention services. METHODS: Both qualitative and quantitative data were collected from caregivers of children aged 0-4 years with SCD across two sites in the United States. Caregivers completed the Knowledge of Infant Development Inventory, a custom survey about their experiences with early intervention, and a qualitative interview. RESULTS: A total of 127 caregivers were approached, 47 participated in surveys, and 20 completed interviews. Caregivers expressed varying levels of confidence and understanding of developmental milestones across sites. Interviews highlighted three main themes: fear of SCD-related complications, variable buy-in to early intervention, and the importance of provider-caregiver relationships. While some caregivers appreciated early intervention, others questioned its necessity. Caregivers communicated interest in connecting with other families facing similar challenges, emphasizing the need for increased awareness of available resources. CONCLUSIONS: Fear about their child's well-being was expressed by many caregivers, emphasizing the need for a supportive healthcare team that can help families connect with preventive interventions. While about a quarter of children had been referred to rehabilitation services, caregivers were unaware of the elevated risk for developmental delay, which diminished caregiver interest in participating in programs like early intervention. This study underscores the importance of addressing knowledge gaps and overcoming barriers to enhance care for families affected by SCD.

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Sickle cell disease includes various inherited hemoglobinopathies due to the production of abnormal hemoglobin molecules. This can lead to significant clinical complications and sequelae. Hemoglobin SC (HbSC) is a notable variant of SCD, requiring early diagnosis and management to prevent severe outcomes. This case report highlights the critical need for SCD screening, particularly among immigrant populations where current U.S. guidelines do not mandate arrival screening. We present the case of a West African male, age 45, with chronic osteomyelitis, who developed a life-threatening pulmonary embolism (PE) due to peripherally inserted central catheter (PICC line) thrombosis, triggering a hemolytic crisis and thereby revealing HbSC disease. The authors of this report advocate for routine SCD screening in high-risk populations through targeted screening programs. Through multidisciplinary management and public health initiatives, we can address the gap in screening and ensure early detection and treatment of SCD in vulnerable populations.

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INTRODUCTION: Sickle cell disease is the most common hereditary hemoglobinopathy followed by beta-thalassemia. Until recently, allogeneic stem cell transplantation was the only curative approach. Based on the Crispr-Cas9-technology enabling targeting specific genes of interest, fetal hemoglobin which is normally shut-off after birth can be switched on and sufficient levels can alleviate symptoms in sickle cell disease and avoid transfusions in beta-thalassemia. Two first-in-human clinical studies in sickle cell disease and beta-thalassemia aiming to increase the level of fetal hemoglobin by using Crispr-Cas9 to modify autologous hematopoietic stem cells in patients aged 12-35 years have proved safety and efficacy and have shown promising clinical outcomes. AREAS COVERED: The paper summarizes the outcome of the results of the two recently published clinical studies and compares them with the other available curative approaches. EXPERT OPINION: Based on the currently available safety and efficacy data of the two published clinical results on gene therapy with Crispr-Cas9 modified autologous stem cells (exagamglogene autotemcel), it can be anticipated that this approach will add significantly to the therapeutic options for patients with sickle cell disease and beta-thalassemia and can be considered for all patients above 12 years of age independent of a suitable allogeneic stem cell donor.

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A patient who had sickle cell disease and had spleen uptake on bone scans is described, and additional causes for that finding are discussed.

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Objective: Sickle cell disease (SCD) is an inherited hematologic disorder that impacts approximately 100,000 Americans. This disease is associated with progressive organ damage, cerebral vascular accident, and neurocognitive deficits. Recent guidelines from the American Society of Hematology (ASH) recommend cognitive screening with a psychologist to help manage cerebrovascular risk and cognitive impairment in this population. SCD patients benefit from neuropsychology services and several institutions already have programs in place to monitor cognitive risk. Program Description: We describe a longitudinal neurocognitive evaluation program at our institution that serves all patients with SCD, regardless of disease severity or referral question. The Sickle Cell Assessment of Neurocognitive Skills (SCANS) program was established in 2012. We outline the program's theoretical framework, timepoints for evaluation, test battery, logistics, patient demographics, integration with research programming, and multidisciplinary collaboration to support optimal outcomes. Program Outcomes: Our program has provided 716 targeted neuropsychological evaluations for patients over the last decade. Nearly 26% of patients in the program have been followed longitudinally. The most common diagnoses generated across cross-sectional and longitudinal evaluations include cognitive disorder (n = 191), attention-deficit/hyperactivity disorder (n = 75), and specific learning disorder (n = 75). Approximately 87% of patients who participated in SCANS during late adolescence successfully transitioned from pediatric to adult care. Conclusion: We discuss considerations for developing programming to meet the needs of this population, including tiered assessment models, timing of evaluations, scope, and reimbursement. Program models that utilize prevention-based tiered models or targeted evaluations can assist with serving large volumes of patients.

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INTRODUCTION: Skull infarction is an uncommonly reported complication of sickle cell disease. We aimed to characterize the clinical and imaging features of skull infarction in pediatric patients with sickle cell disease. METHODS: We searched the PubMed database for case reports on skull bone infarction in pediatric patients with sickle cell disease. Out of 67 records retrieved, 15 met inclusion criteria, and a 16th case reported by the senior author was included. We extracted and analyzed clinical and imaging data. RESULTS: The most common symptom at onset was headache (88%). Bilateral skull infarction (50%) and parietal bone involvement (82%) were frequent imaging findings. Epidural hematoma developed in 65% of the cases, 30% of patients required drainage, and exchange infusion was reported in 18%. No fatal outcomes were reported. CONCLUSIONS: Skull infarction is a potentially severe complication of sickle cell disease presenting unique clinical challenges. Acute headaches should raise suspicion for this condition and may require additional investigation.

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Objectives The study sought to evaluate and compare the maternal and fetal outcomes of pregnancy in women with sickle cell disease (SCD) versus healthy pregnant women in Bahrain. The objective was to update the available data in order to come up with a strategy to implement a multidisciplinary management program, which will enhance pregnancy outcomes for the SCD patient population. Materials and methods This retrospective case-control study was conducted in the Obstetrics and Gynecology Department at Salmaniya Medical Complex (SMC) in Bahrain. The study group consisted of all pregnant women with homozygous SCD (HbSS) who delivered at SMC between January 1, 2019, and December 31, 2021. The control group comprised pregnant women who delivered at SMC during the same period but did not have SCD or trait. Data for the study were collected from the healthcare system records at SMC, specifically the I-Seha electronic medical record system and the labor room registry book. A thorough review and analysis of the data were conducted, encompassing 217 cases of SCD and 200 controls. The variables examined included nationality, age, gravidity, parity, gestational age, reason for admission, antenatal/postnatal complications (such as urinary tract infection, pneumonia, acute chest syndrome, thromboembolism, premature rupture of membranes, hypertension, pre-eclampsia, and intrauterine growth restriction), type of delivery, birth weight, newborn outcome, and postnatal complications. Results Pregnant women with SCD experienced significantly higher rates of antenatal hospitalization compared to controls - 69.6% were admitted at least twice versus only 16.5%. Vaso-occlusive crises were the primary reason for admission in over half of SCD patients, with 22.6% having one episode, 11.1% having two, and 20.3% having more than two during pregnancy. Low hemoglobin levels also necessitated admission in 11.1% of SCD women, while no controls required hospitalization for this. The burden of maternal morbidity was substantially greater in the SCD group, with only 20.3% free of complications versus 94% in controls. SCD women had elevated rates of blood transfusions, acute chest syndrome, and urinary tract infections. Adverse pregnancy outcomes were also more common, including higher risks of preterm birth, low birth weight, and intrauterine growth restriction. Despite these increased maternal and fetal risks, there was no significant difference in the incidence of hypertensive disorders between groups. Interestingly, our data showed a significantly lower incidence of gestational diabetes in the SCD group compared to controls (8.3% vs. 18%). Tragically, one maternal death occurred in the SCD group, although the overall maternal mortality did not differ significantly. Conclusion SCD poses substantial risks for mother and fetus. Careful monitoring with a multidisciplinary team and patient education are crucial. Early detection can reduce morbidity and mortality. Further research is needed on interventions to improve outcomes.

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BACKGROUND: Sickle cell disease (SCD) is a prevalent genetic disorder characterized by abnormal hemoglobin formation, resulting in severe complications. Hydroxyurea (HU) therapy has demonstrated efficacy in reducing SCD-related complications; however, its utilization patterns and patient perceptions remain underexplored, particularly in the Al Ahsa region of Saudi Arabia. OBJECTIVE: This cross-sectional study aimed to assess the prevalence of HU usage among adult patients with SCD in Al Ahsa; identify the barriers to starting, maintaining, and discontinuing HU therapy; and evaluate the patient-reported outcomes associated with its use. METHODS: Data were collected through face-to-face surveys and medical record reviews of adult SCD patients attending outpatient clinics in the Hereditary Blood Diseases Center of Al Ahsa, Saudi Arabia, between December 2023 and March 2024. Descriptive statistics and inferential analyses were performed using SPSS version 26. RESULTS: A total of 345 adult SCD patients were included, with a mean age of 34.12 ± 11.1 years. Most participants were male (58.6%) and unmarried (55.4%). HU utilization was reported by 57.1% of the participants, with the highest adherence observed among older age groups (p = 0.001). Significant improvements in pain severity, hospitalization rates, and quality of life were reported among HU users (p < 0.001). Common barriers to HU use included concerns about side effects, lack of medical justification, and absence of medical advice. CONCLUSION: This study provides valuable insights into the utilization and perceptions of HU therapy among adults with SCD in Al Ahsa, Saudi Arabia. Addressing identified barriers and promoting patient education are crucial for optimizing therapy adherence and improving clinical outcomes in this population.

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BACKGROUND: Priapism is a urological condition characterized by a persistent erection. The management varies based on its subclassifications. Despite established clinical guidelines for ischemic priapism, there is a lack of large-scale research focused on patient characteristics and management strategies. OBJECTIVES: To analyze the contemporary management of ischemic priapism in the US, exploring patient demographics and clinical characteristics, as well as predictors of erectile dysfunction (ED) and penile prosthesis implantation (PPI). MATERIALS AND METHODS: We performed a retrospective analysis of the PearlDiver Mariner database, reviewing records from 2010-2021. Adult males diagnosed with ischemic priapism were included. Data analysis covered demographic, clinical variables, and management strategies. Predictors of de novo ED and PPI were evaluated using multivariable logistic regression analysis. RESULTS: Of 36,120 patients, most (93%) received only medical management, and a minority underwent surgical interventions (penile shunt surgery [PSS], PPI or both). Medical management was typically effective, as 67.08% of the patients in this group experienced only one episode of priapism. However, de novo ED occurred in 16.57% of these patients. The majority of patients undergoing PPI had an inflatable prosthesis (81%). Older age (odds ratio, OR 1.02), the presence of metabolic diseases (OR 1.39), neurogenic disorders (OR 1.72), solid pelvic malignancies (OR 1.09), and multiple episodes of priapism were identified as significant predictors of de novo ED (all p < 0.05). Similarly, age (OR 1.03), the presence of metabolic diseases (OR 1.23), solid pelvic malignancies (OR 1.99), and multiple episodes of priapism were associated with higher likelihood of PPI (all p < 0.05). CONCLUSION: Most cases of ischemic priapism are managed with the medical therapy. Less than 3% of patients with ischemic priapism receive PPI, and when this occurs an inflatable prosthesis is favored. Age, specific comorbidities, and multiple episodes of priapism appear to be significant predictors of ED and PPI.

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Due to the significant morbidity and mortality of hemoglobinopathies, curative options have long been pursued. The overall goal of gene therapy is to modify a patient's own hematopoietic stem cells to overcome the deleterious effects of the underlying genetic defect by gene addition, gene editing, or gene silencing. Gene addition incorporates genes with superior function than the abnormal gene; gene editing takes advantage of molecular tools such as zinc finger proteins, Transcription Activator-Like Effector Nucleases and Clustered Regularly Interspaced Short Palindromic Repeats coupled with Cas9 proteins (CRISPR-Cas9) which allow for sequence-specific breaks in DNA that disrupt gene function; and gene silencing suppresses gene expression by interference with mRNA transcription/protein translation or epigenetic modification. The majority of gene therapy strategies for hemoglobinopathies have targeted erythroid-specific BCL11A, a major regulator of fetal hemoglobin repression at the gamma-globin locus, in the normal fetal-to-adult hemoglobin switch that occurs shortly after birth. Other goals have involved the incorporation of anti-sickling globins, such as ßT87Q or ßAS3. Landmark clinical trials of gene therapy in transfusion-dependent thalassemia and sickle cell disease have shown remarkable efficacy and acceptable safety and culminated in recent regulatory approvals of gene therapy for both diseases in Europe and the United States.

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RATIONALE: Cardiopulmonary dysfunction is a major contributor to mortality among persons with sickle cell disease (pwSCD). Despite this, little is known regarding environmental drivers of lung function decline. OBJECTIVE: We hypothesized that environmental and socioeconomic variables have a significant effect on lung function in pwSCD that can be detected by spirometry. METHODS: We retrospectively analyzed all spirometry results from pwSCD followed in the Pediatric Pulmonology clinic at the Children's Hospital of Philadelphia since 1 January 2016. RESULTS: The study included 349 spirometry tests from 128 patients, primarily "Black or African American" (88%) and male (61%). More frequent exposure to PM2.5 above 25 µg/m3 was associated with higher odds of obstruction. Specifically, when compared to incidence of exposure to PM2.5 above 25 µg/m3 <25th percentile, both pwSCD exposed to 25th-75th percentile and pwSCD >75th percentile had higher odds of obstruction on spirometry (25th-75th: odds ratio [OR]: 9.6, p = .017; >75th: OR: 31.85, p = .002) despite correction for potential confounders. Similarly, median household income below the mean was associated with higher odds of restriction (OR: 4.37; p = .009). CONCLUSIONS: We report higher odds of obstruction in pwSCD frequently exposed to PM2.5 concentrations above 25 µg/m3 and higher odds of restriction in pwSCD with lower household income. Our findings link spirometry patterns to modifiable risk factors indicating that there may opportunities for early intervention in pwSCD that have been referred to a pulmonology clinic. Further research is needed to assess if these findings can be generalized to the wider population of pwSCD.

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Background: Stuttering priapism is recurrent, self-limited episodes of sustained penile erection and is common in patients with sickle cell disease (SCD). Prevention of stuttering priapism is important to avoid progression to episodes of ischemic priapism which can cause erectile dysfunction. Priapism has been shown to be associated with increased nocturnal hypoxemia in patients with SCD. Case Description: A 43-year-old male with nocturnal episodes of stuttering priapism that was refractory to treatment with multiple medications was found to have obstructive sleep apnea (OSA). Following treatment of this condition with a continuous positive airway pressure (CPAP), the patient had immediate symptom relief and has had three months without an episode of priapism. Conclusions: OSA should be considered as an underlying cause of nocturnal stuttering priapism in patients with SCD, particularly in patients who present with stuttering priapism later in life or patients who present strictly with nocturnal episodes. Appropriate management of OSA can significantly decrease the incidence of stuttering priapism in patients with SCD.

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OBJECTIVES: High rate of alloimmunization in sickle cell disease (SCD) patients poses a significant challenge in finding compatible blood unit. Accurate determination of the blood group genotype of them can help reduce the alloimmunization risk. Tetra ARMS PCR is a novel method that has been utilized recently to investigate SNPs in diseases in a fast and reliable way. METHODS: Our study included 104 SCD and sickle thalassemia (Sß) patients referred to Baghaei-2-Hospital of Ahvaz in 2019 using a nonrandom sampling method. Blood samples were collected for serological and molecular tests. Rh genotyping was performed using Tetra ARMS PCR and compared with the serological results. RESULTS: Based on the Tetra ARMS PCR method, out of 104 patients, 7 (6.7%) were d/d, 40 (38.5%) were D/d, 57 (54.8%) were D/D, 25 (24%) were C/C, 59 (56.7%) were C/c, 20 (19.3%) were c/c, 4 (3.8%) were E/E, 25 (24%) were E/e, and patients 75 (72.2%) were e/e. There were discrepancies in the serological and molecular results for 11 patients. CONCLUSION: Use of Tetra ARMS PCR in combination with serological methods for determining the Rh blood group system in donors and transfusion-dependent patients represents a remarkable transformation in the field of immunohematology.