Sickle cell disease (SCD) is a common genetic disorder affecting up to 2.6% of the population in Saudi Arabia. SCD results in severe disability, reduced quality of life, extensive use of medical resources, increased economic burden, and a high likelihood of increased mortality. Red blood cell transfusion remains a cornerstone in the management of SCD complications. This position paper highlights the current state of SCD management within the Kingdom of Saudi Arabia. Despite the advantages of automated red blood cell exchange (aRBCX) and guideline recommendations, its use remains limited. In practice, aRBCX is used for a variety of indications, including acute management and prophylaxis of stroke, systemic fat embolism, severe forms of acute chest syndrome, preoperative management, hematopoietic stem cell transplantation, hepatic crisis, and priapism. However, aRBCX is underutilized in pregnancy. Common gaps identified by the advisory panel include the absence of standardized national guidelines, limited access to aRBCX, issues with vascular access, lack of equipment, and insufficient staff training. Another limitation to the use of aRBCX is the higher blood requirements compared to other blood transfusion modalities. These factors contribute to geographical disparities in the management of SCD and suboptimal patient outcomes. To address these issues, the advisory panel recommended developing and implementing evidence-based national guidelines, expanding access to aRBCX, enhancing health staff education and training, and establishing a robust national SCD registry. By prioritizing these recommendations, we can help streamline SCD care, reduce practice variation, and nationalize sickle cell disease management in Saudi Arabia to improve patient care.

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 COVİD-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: Vaso-occlusive and hemolysis are the clinical hallmarks of sickle cell disease .patients with sickle cell at risk of iron overload due to chronic blood transfusion treating complications of the disease ,in addition to increasing iron absorption from gut and chronic hemolysis. Material and Methods: This is retrospective study on total of 174 adult sickle cell disease patients were included in this study, attending hematology clinic in king Fahad Hofuf hospital and inherited blood disorder center-Alhassa. for each patient demographic data was obtained (age, sex, nationality), history of previous blood transfusion, hemoglobin level, serum ferritin level. In total, 174 naïve Saudi adult patients (113 males, 61 females) were included in this study. Mean age was 28.2 ±10 years (range 12- 62 years), sex ratio (M/F) was 1.8 :1.0 . 93 (53%) patients had history of previous blood transfusion. The overall mean serum ferritin concentration was 587 ± 547(18.49 - 2660 ng\dl). The mean serum ferritin level for male is 649 ± 606 ng\dl and mean serum ferritin level for female is 473± 440ng\dl. Conclusion: Iron overload in sickle cell disease patients from eastern province remains mild to moderate, with significant sex difference. The repetitive assessment of serum ferritin level should be consider to sickle cell patients whom frequent blood transfusion is needed like stroke and renal failure.

Background: Blood transfusion is a key treatment of sickle cell disease (SCD) complications. Delayed hemolytic transfusion reaction (DHTR) is a delayed reaction, that occurs days to weeks following a transfusion, characterized by mild anemia and/or hyperbilirubinemia and is one of the serious complications of blood transfusion. The symptoms of DHTR resemble those of vaso-occlusive crisis secondary to SCD leading to difficulty or delaying in diagnosis of DHTR. DHTR may lead to multiple organ failure and death. Case Report: A 31-year-old female patient with a known case of SCD presented to our ER in King Fahad hospital Hofuf in the Kingdom of Saudi Arabia, with a history of generalized body ache, exertional dyspnoea, headache and easy fatigability for a few days on a background history of episodic hospital admissions for SCD, but she was admitted 3 times over the previous 6 months and received 6 units of packed red blood cells (PRBCs). The last blood transfusion was 18 days earlier. She was sick and her Hb level was 4.5 g/dL with positive Coombs test and positive alloantibodies, diagnosed as DHTR. We treated her with prednisolone tablets 1 mg/kg daily, intravenous immunoglobulins, 0.4 gm/kg daily for 5 days, and rituximab 500 mg IV every week for 4 weeks. Her Hb level raised up to 8.2 g/dL and she was discharged in good condition. Conclusion: Identifying risk factors for DHTR by history and presentation is urgently needed in order to risk stratify the transfusion regimen. It is important to avoid additional transfusions in these patients if possible because these may exacerbate the hemolysis and worsen the degree of anemia.

Sickle Cell Anemia is a genetic disorder caused by abnormal hemoglobin S production, leading to sickle-shaped red blood cells, anemia, and high morbidity and mortality rates. In Saudi Arabia, sickle-cell mutation affects 4.45% of the population, with a mortality rate of 0.73%. This case-controlled study aimed to identify mortality-associated factors among adults with Sickle Cell disease in Al-Ahsa from 2018 to 2022. Univariate analysis revealed several independent risk factors for mortality, such as gender, older age, chronic disease, SCD complications, previous surgery, CXR infiltration, and positive blood culture. Males exhibit a higher risk of non-survival compared to females, potentially due to more severe symptoms and complications. Older patients (>35 years) face an increased risk of non-survival due to age-related end-organ damage. Comorbidities, such as renal diseases and bronchial asthma, are prevalent in SCD patients and worsen with age. Lower hemoglobin levels, higher hospitalization frequency, and vaso-occlusive crises are associated with increased mortality rates. The study emphasizes the importance of comprehensive care, including specialist access, regular follow-up, and proper treatment, to improve survival outcomes. Early recognition and management of complications, such as renal failure and infection, along with interventions like vaccinations, prophylactic antibiotics, hydroxyurea, and splenectomy, can positively impact survival. Further research is needed to explore the underlying molecular mechanisms and develop more effective treatments for SCD.