Sickle cell disease (SCD) is the most common monogenic disorder, although the diversity and heterogenicity of clinical presentations render estimations of disease severity unpredictable. This cross-sectional study aimed to determine if laboratory markers could serve as indicators of SCD severity. We enrolled 90 adult patients with SCD with a mean age of 32.33 ± 11.84 years from the eastern province of Saudi Arabia, where SCD is more common than in other regions. Our study revealed a positive significant association between the number of hospitalizations and emergency visits with white blood cells (WBC) (R = 0.241, R = 0.207), respectively. Similarly, positive significant associations were found between the number of hospitalizations and emergency visits with platelets (R = 0.393, R = 0.276), respectively. Conversely, negative significant relationships were found between the number of hospitalizations and emergency visits (ER) with hemoglobin (Hb) F (R = -0.268, R = -0.263), respectively. Additionally, significant negative relationships were found between Hb F (R = -0.223) and the frequency of ICU admission. Only the number of hospitalizations and emergency visits annually were significantly predicted with P values of 0.021 and 0.038, respectively. Moreover, an increase in WBC was found to significantly increase the chance of undergoing splenectomy by 23.02%. SCD is a multisystemic disease with heterogeneous clinical presentations and disease severity. Inflammatory markers are valuable tools for better risk stratification and could be translated into developing new therapeutic strategies and modifying the treatment paradigm.
Anemia, Sickle Cell , Severity of Illness Index , Humans , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/epidemiology , Anemia, Sickle Cell/diagnosis , Cross-Sectional Studies , Adult , Male , Female , Saudi Arabia/epidemiology , Biomarkers/blood , Hospitalization/statistics & numerical data , Young Adult , Middle Aged , Emergency Service, Hospital/statistics & numerical data
INTRODUCTION: Vaccination has a fundamental role in protecting against and modifying the severity of several infectious diseases, including COVID-19. Several immune and non-immune adverse events have been reported post-COVID-19 vaccine. The aim of this study was to assess the effect of the COVID-19 vaccine on women's menstrual bleeding. METHODS: A cross-sectional study was conducted among 399 vaccinated women in the Eastern Province of Saudi Arabia. Data were collected using a direct interview-based questionnaire including four sections. RESULTS: A total of 399 women were recruited, with a mean age of 25.54 ± 6.177 years. More than half (53.9%) of the participants reported post-vaccination menstrual bleeding abnormality in terms of a heavy or irregular cycle. Out of the total number, 40 (21.4%) women reported having heavy menstrual flow, and 67 (16.8%) had non-menstrual bleeding after receiving the COVID-19 vaccine. Fully vaccinated women were at a greater risk of post-vaccination menstrual bleeding abnormality (p = 0.058). However, there was no correlation between booster shot type and menstrual bleeding abnormality (p > 0.05). In addition, there was no significant association between non-menstrual bleeding and the type of booster shot, the type of the first dose, and prior history of COVID-19 infection (p > 0.05). CONCLUSION: Despite vaccination being the most effective way to prevent COVID-19, it does have an impact on menstrual bleeding in terms of menorrhagia and metrorrhagia. Therefore, more studies are needed to understand the mechanism and the long-term impact of COVID-19 vaccines on the hemostatic system.
Purpose: Sickle cell disease (SCD) is a multisystemic disease that results in diverse clinical manifestations in the form of acute and chronic complications. This study aims to assess the academic achievements of patients with SCD. Methods and Materials: A cross-sectional study was a study conducted among adult patients with SCD from the eastern province of Saudi Arabia, where SCD is more widespread than in other regions. Results: A total of ninety patients with SCD, whose median age was 32.33 ± 11.84, were retrospectively evaluated. Of the total number, 32 (35.5%) did not obtain a secondary higher education certificate, and five (3.3%) were illiterate. Sixty-three (70%) of the patients were female, indicating no significant association between gender and education level (p-value > 0.05). The patients' mean annual hospitalizations and emergency visits totaling 4.7 and 8.43, respectively, were not significantly associated with education (p-value > 0.05). Throughout the patients' lives, mean blood transfusions and ICU admissions occurred 6.29 and 2.75 times, respectively, which were not significantly associated with education level (p-value > 0.05). However, education was inversely proportional to a history of splenectomy and cholecystectomy (p < 0.05). Laboratory parameters (hemoglobin level, WBC, platelets, and hemoglobin F) showed no significant associations with education level (p-value > 0.05). Patients with sickle-thalassemia exhibited a trend of being better-educated than those without the diseases, but the difference did not reach statistical significance (p-value > 0.05). A linear regression analysis revealed no significant associations among clinical and laboratory parameters. Conclusion: SCD is a multisystemic disease that impacts socioeconomic status, learning ability, and academic achievements. More research is needed to identify factors that predict poor performance. Additionally, intelligence quotient (IQ) and cognitive assessment should be incorporated into early screening programs for SCD in order to implement preventive measures and a thorough investigation of underlying causes.
Introduction: We report a case of new-onset thrombocytopenia following administration of a loading dose of ticagrelor. Case Presentation: A 66-year-old male known to have diabetes mellitus type II, chronic obstructive airway disease, and hypertension presented to the emergency department with retrosternal chest pain and dyspnea. Work-up on presentation showed Hb 14.7 g/dL, platelet 229 × 109/L, and troponin 309 ng/mL. The electrocardiogram showed ST elevation in the anterior-lateral leads. The patient underwent balloon angioplasty, and a drug-eluting stent was deployed. During the procedure, intravenous unfractionated heparin and a 180 mg loading dose of ticagrelor were given. Six hours post procedure, the platelet count was 70 × 109/L without active bleeding. Blood smear was unremarkable, and no schistocytes could be seen. So, ticagrelor was stopped, and the patient's platelet count completely recovered four days after discontinuation. Conclusion: Ticagrelor-induced thrombocytopenia is a rare but increasingly recognized entity. Therefore, post-treatment monitoring and early recognition are crucial parts of management.
Background and Aim: Sickle cell disease (SCD) is an autosomal recessive disorder. Decisions following premarital screening results might be influenced by several factors. Thus, this study aims to assess the knowledge and beliefs toward SCD and reproductive decisions. Material and Methods: A cross-sectional study was conducted among adults in the eastern province of Saudi Arabia. Data was collected using a web-based questionnaire. Participants were divided into two groups based on their knowledge scores. Results: A total of 390 participants were recruited with a mean age of 28.6 years. The majority (90.5%) of participants had general knowledge about the disease mode of inheritance however, 66.9% had poor knowledge about the disease overall. Regarding knowledge level, there was no statistically significant difference between the non-healthy group (diseased and carrier) and healthy participants (P=0.304). Moreover, the participants' decisions about choosing future partners were more likely to be affected among those with prior knowledge about the disease (p=0.008). However, 152 (91.6%) male participants with prior knowledge would change their decision about selecting a future partner compared to 225 (92.4%) female participants. Regarding reproduction, only 38.5% of participants were aware of in vitro fertilization (IVF) as an assistive reproductive technology. Furthermore, female participants believed that IVF is a way to have healthy babies and would consider it despite the cost more than male participants (p=0.0001, p=0.007 respectively). Conclusion: SCD is an inherited disease with economic, physical, and psychological burdens. However, curative options are costly, and hence, prevention is key. Therefore, healthcare decision-makers should consider implementing policies to minimize the financial burden that may still affect society despite the availability of free medical care. This study warrants extensive community-based education programs that may contribute toward cost savings. It also highlights the importance of premarital counselling for disease and carrier people including alternative reproduction options.
COVID-19 is a severe respiratory disease with a spectrum of clinical presentations and complications. Warm autoimmune hemolytic anemia (WAIHA) is increasingly recognized in patients with COVID-19 either while infected or shortly after infection. We report a 36-year-old male with clinical and laboratory findings consistent with WAIHA. His medical history was significant for COVID-19 infection three months before presentation. He was initially resistant to steroids but had substantial improvement following initiation of rituximab, with complete recovery thereafter. Therefore, serial assessment of complete blood cell count parameters and hemolysis markers post-COVID-19 infections is warranted for early detection and prompt treatment.
Background and Aims: Thrombocytopenia is increasingly recognized among patients with critical illness and plays a role in several diseases affecting different organ systems. Therefore, we studied the prevalence of thrombocytopenia among hospitalized COVID-19 patients and its correlation with disease severity and clinical outcomes. Methods: This was an observational retrospective cohort study conducted on 256 hospitalized COVID-19 patients. Thrombocytopenia is defined as a platelet count below 150,000/µL. Disease severity was classified based on the five-point CXR scoring tool. Results: Thrombocytopenia was found in 66 (25.78%) patients. In outcomes, 41 (16%) patients were admitted to intensive care unit, 51 (19.9%) died, and 50 (19.5%) had acute kidney injury (AKI). Of the total patients with thrombocytopenia, 58 (87.9%) had early thrombocytopenia, while 8 (12.1%) had late thrombocytopenia. Notably, mean survival time was markedly decreased in late-onset thrombocytopenia cases (p < 0.0001). Patients with thrombocytopenia showed a significant increase in creatinine compared to those with normal platelet counts (p < 0.05). Moreover, thrombocytopenia was more prevalent in patients with chronic kidney disease compared to other comorbidities (p < 0.05). In addition, hemoglobin was significantly lower in the thrombocytopenia group (p < 0.05). Conclusion: Thrombocytopenia is a common finding among COVID-19 patients, with a predilection toward a specific group of patients, though the exact reasons are unclear. It predicts poor clinical outcomes and is closely linked to mortality, AKI, and the need for mechanical ventilation. These findings suggest that more research is required to study the mechanism of thrombocytopenia and the possibility of thrombotic microangiopathy in COVID-19 patients.
Purpose: Sickle cell disease (SCD) comprises a complex group of hematologic disorders that are collectively the most common monogenic disorder and are associated with increased risk of intensive care unit admission (ICU). The purpose of this study is to investigate factors that predict admission of adult patients with SCD to the ICU. Patients and Methods: This was a cross-sectional study that enrolled adult patients with SCD from Saudi Arabia. Results: A total of 107 patients with SCD, with a median age 31.9±12.1 years, were evaluated retrospectively. Regarding predictors of ICU admission, patients who indicated a history of blood transfusions were at 8.047-fold higher risk of ICU admission (OR=8.047; 95% CI=2.392-27.07; p=0.001). Patients who started hydroxyurea were at least 3.071 times more likely to be admitted than those who did not (OR=3.071; 95% CI=1.164-8.104; p=0.023). We also observed three or more hospitalizations per year to be associated with increased risk of ICU admission (OR=3.393; 95% CI=1.285-8.960; p=0.014), with those making 3 to 5 visits annually having at least 10.4 times higher risk (OR=10.38; 95% CI=10.098-98.19; p=0.041) and those with 6 to 10 ER admissions having 18 times higher risk (OR=18.00; 95% CI=2.149-150.8; p=0.008). Finally, patients with high WBC were predicted to have at least 3.34 times higher risk of ICU admission (OR=3.337; 95% CI=1.131-9.846; p=0.029). Conclusion: SCD is a multi-systemic disease associated with increased morbidity and mortality. Recognition of high-risk features in patients helps to eliminate subjectivity in ICU referral decision. Frequent hospitalization and emergency visits, multiple blood transfusions, and elevated white blood cell count were significantly associated with a higher rate of ICU admission despite hydroxyurea usage.
Background: MyD88-adapter-like (MAL), as an essential adapter protein for a variety of TLRs (Toll-like receptors), modulates the inflammatory response. Many infectious illnesses are influenced by single nucleotide polymorphisms (SNPs) that modify MAL function. We aimed to examine the influence of the MAL rs8177374 polymorphism on Plasmodium falciparum malaria susceptibility and severity. Patients and Methods: Samples from 141 Plasmodium falciparum malaria patients and 147 healthy controls were used in the study. Patients were subdivided into mild and severe groups based on their clinical results, as defined by the World Health Organization (WHO). Genotypes for MAL rs8177374 were identified by allele-specific PCR technique, and TNF-alpha and IL-12 levels were measured using ELISA. Results: The MAL rs8177374 (CT) genotype is associated with an increased risk of malaria (OR: 2.52; 95% CI: 1.44-4.41). Furthermore, the CT and TT genotypes gave considerable protection against severe malaria (OR: 0.07; 95% CI: 0.03-0.19 and OR: 0.03; 95% CI: 0.007-0.1 respectively). And the T allele was linked to a higher risk of malaria (OR: 1.7; 95% CI: 1.18-2.5), while protecting patients from severe malaria (OR: 0.135; 95% CI: 0.07-0.3). Mutants (CT and TT) have greater TNF-alpha and IL-12 levels compared to wild-type (CC). Conclusion: Malaria risk is linked to single nucleotide polymorphism in the MyD88-adaptor-like gene. People with the MAL rs8177374 mutant variant may be less likely to get severe malaria.
Objectives: Splenectomy is considered a therapeutic modality for several hematological diseases, although complications are possible. This study assessed the effects of splenectomy on various hematological disorders and the roles of prophylactic measures on postoperative outcomes. Methods: This was a cross-sectional study performed in KSA on adult patients with underlying non-malignant hematological disorders who had undergone splenectomy. Results: This study examined 179 patients with various hematological disorders, 38 (21.1%) of whom had undergone a splenectomy. Of those 38 patients, more than two-thirds (73.7%) had an open splenectomy. The average hospital stay was 2-7 days, and no significant difference was observed between the open and laparoscopic approaches. Approximately 95% of the patients showed overall improvements in their condition after splenectomy. However, 26.3% of patients reported a recurrence or need for further treatment 1 year or more after splenectomy. Approximately 16% of patients had an increased incidence of postoperative infectious complications, particularly patients with sickle cell disease and beta thalassemia. More than half the patients who developed complications had not received vaccination preoperatively, whereas 44.4% of vaccinated patients experienced complications (p = 0.04). Conclusion: Splenectomy is considered a universal line of treatment for most non-malignant hematological diseases. Although splenectomy is an effective treatment, the reasons why patients with the same disease can have different responses remains unclear. Infection is a common postoperative complication, and vaccinations are underused. This study emphasizes the roles of patient education, scheduled vaccinations and proper selection of patients in the use of splenectomy for the treatment of non-malignant hematological diseases.
Primary myelofibrosis and paroxysmal nocturnal haemoglobinuria (PNH) are uncommon clonal blood disorders that are rarely found together. We report a case of primary myelofibrosis (PMF) with concomitant subtle PNH in a 42-year-old man who presented with a 4-week history of fatigue, unexplained chest pain, and new-onset erectile dysfunction. Bone marrow biopsy showed severe fibrosis consistent with PMF. However, smooth muscle dystonia symptoms in the form of new-onset erectile dysfunction and oesophageal spasm were not fully explained by PMF but were clues for PNH, confirmed by flow cytometric assays. Routine PNH testing for patients with new-onset PMF and clinical symptoms suggestive of PNH, as well as those with refractory anaemia despite effective therapy, is crucial since these two conditions can coexist. As a result, a lack of early testing may cause a delay in diagnosis, increasing the patient's transfusion load and the facility's costs.
BACKGROUND AND AIM: Hydroxyurea (HU) plays an essential role in the complex pathophysiology alteration of sickle-cell disease (SCD), which translates clinically into the enhanced quality of life and increased survival. This research examines adult patients with SCD's attitudes and awareness toward HU, as well as underutilization consequences. METHOD: A cross-sectional research was performed in Saudi Arabia, and adult patients with SCD were interviewed. The survey includes patient demographics, attitudes, and knowledge of HU and clinical data. The chi-square was applied through SPSS version 23 for assessing any association with outcome variables and demographic characteristics. RESULTS: HU is known to 72 (67.3%) of the 107 patients. The hydroxyurea treatment was initiated in 46 patients (63%). Of these, 23 (50%) discontinue HU, with the key factors being pregnancy preparation and side effects development. For those who were unaware of HU, 13 (37.1%) were admitted to the intensive care unit because of acute chest syndrome, 29 (82.8%) required a frequent blood transfusion and 12 (34.2%) with frequent hospitalizations. However, there was no significant relationship between awareness and education level (P value is .078 > .05). In addition, there was no significant relationship between the level of awareness and age and gender of participants (P value is .68 and .44, respectively). CONCLUSION: HU is a long-established and effective disease-modifying agent for SCD patients, but it is underutilized. The causality of underuse is bidirectional between patients and healthcare providers. It is essential to educate healthcare providers and patients with SCD about hydroxyurea role in modifying disease severity, resolving adverse events, and achieving full benefits.
BACKGROUND: Heavy menstrual bleeding (HMB) is a common clinical problem. However, seeking medical advice might be delayed until patients develop several clinical consequences. AIM: To assess the prevalence and awareness of HMB among the Saudi female population and measures that are commonly used to control the bleeding. METHOD: This is a cross-sectional study where a trained study team member carried out a survey. The survey includes patient demographics and medications history, comorbidities, blood transfusion, and patient perception about her period. HMB was defined as bleeding lasting >7 days, flooding or changing protection more than every 2 hours, and passing clots >1 in. in diameter. RESULTS: Four hundred and thirty-one women were evaluated for HMB with a median age of 27.72 ± 7.75. Out of the total number, 281 (65.2%) females were identified to have HMB. Among these 281 females, only 35.6% were aware of having HMB, whereas 64.4% either unaware or unsure about having it. For those who were aware of having HMB, only 32% seek medical advice, particularly gynecology clinic though 46.6% were not satisfied with offered management. On the other hand, 28% and 3.9% of those unaware of having HMB did required iron supplementation and blood transfusion, respectively. CONCLUSION: HMB is a major clinical problem though awareness about having HMB lacks regardless of the age and education level. Raising awareness and a multidisciplinary approach may result in early diagnosis, early intervention, and reduction of clinical consequences.
