Sickle cell disease (SCD) is associated with substantial morbidity and early mortality in afflicted adults. Cardiopulmonary complications that occur at increased frequency in SCD such as pulmonary embolism, pulmonary arterial hypertension, and acute chest syndrome can acutely worsen right ventricular function and lead to cardiogenic shock. Mechanical circulatory support including venoarterial extracorporeal membrane oxygenation (VA ECMO) is being increasingly utilized to treat hemodynamic collapse in various patient populations. However, a paucity of literature exists to guide the use of mechanical circulatory support in adults with SCD where disease-related sequela and unique hematologic aspects of this disorder may complicate extracorporeal therapy and must be understood. Here, we review the literature and describe three cases of adult patients with SCD who developed cardiogenic shock from acute decompensated right heart failure and were treated clinically with VA ECMO. Using an in vitro ECMO system, we investigate a potential increased risk of systemic fat emboli in patients with SCD who may be experiencing vaso-occlusive events with bone marrow involvement given the high-volume shunting of blood from venous to arterial systems with VA ECMO. The purpose of this study is to describe available extracorporeal life support experiences, review potential complications, and discuss the special considerations needed to further our understanding of the utility of VA ECMO in those with SCD.

Management of sickle cell disease complications in the setting of the coronavirus disease 2019 (COVID-19) pandemic is complicated with little published pediatric data. We report the first documented case of a 9-year-old boy with sickle cell disease, presenting with fever, cough, and shortness of breath, diagnosed to have acute chest syndrome and coronavirus disease 2019 (COVID-19) pneumonia with inflammatory storm requiring ventilation, exchange blood transfusion, immunomodulatory agents, and prophylactic anticoagulation. The patient responded satisfactorily to the management of the acute illness and was found to be well at the next visit to the pediatric hematology outpatient department following hospital discharge.

This report documents the treatment of a 41-year-old male with sickle cell disease (SCD) and repeated stuttering priapism using crizanlizumab, which alleviated the priapism but induced a significant vaso-occlusive crisis during the second infusion. Encouragingly, no subsequent vaso-occlusive crises occurred. However, the potential for infusion-related adverse events warrants close supervision. Further research is necessary to explore its full benefits on priapism management.

Sickle cell has predominantly been a disease in the sub-Saharan Africa. There is scanty data in Africa and none in Tanzania regarding patients with sickle cell disease infected with COVID-19 especially in the paediatric group. This is concerning because a large population of children living with sickle cell disease are found in this part of the world where scarcity of resources also prevail. This is the first paediatric case of sickle cell disease and COVID-19 reported in Tanzania; highlighting the presentation and challenges faced in management. It is also the first case in literature describing secondary infection in a child with COVID-19 and underlying sickle cell disease. Our patient was a female child of African origin with underlying sickle cell disease who had recurrent admissions. She presented with cough, fever, chest and abdominal pain and was managed for acute chest syndrome and vaso-occlusive crisis. During the second admission, she developed respiratory distress, infection with COVID-19 was confirmed and managed accordingly. However, she was re-admitted due to bilateral arm pain and physical findings were suggestive of secondary respiratory bacterial infection. She was then started on a different treatment plan. Despite challenges faced in the management of the patient, the outcome was favourable. It is important to recognize the presentation of COVID-19 in children with sickle cell disease and challenges faced in management so that the epidemiologic characteristics, spectrum of the disease and its outcomes can be understood better in the context of sub-Saharan Africa.

Sickle cell anemia is a chronic and debilitating hemoglobinopathy characterized by the presence of abnormal hemoglobin, resulting in the formation of sickle-shaped red blood cells. This case report presents an unusual case of a 32-year-old female patient with sickle cell anemia who had not experienced any previous sickle cell crises since her diagnosis at the age of four years. Despite a stable clinical history, the patient\'s condition rapidly deteriorated, leading to septic shock, multiorgan failure, and atypical complications such as neurological impairment and acute kidney injury. Intensive management strategies, including blood exchange, mechanical ventilation, and aggressive antibiotic therapy, were implemented but unfortunately failed to reverse the progressive clinical deterioration. This case underscores the importance of early recognition and a multidisciplinary approach in managing atypical sickle cell crises to optimize patient outcomes. Further research is needed to improve our understanding and management of such presentations.

Sickle cell disease (SCD) is one of the most common hematological diseases, which results in variable complications. The treatment of SCD is evolving but limited options are available for now. Acute chest syndrome (ACS) is one of the serious complications observed in SCD and a challenging one in prevention. Crizanlizumab is a monoclonal antibody that binds to P-selectin and improves blood flow by preventing sickle cell adhesion to endothelium, resulting in improvement of vaso-oclusive crises (VOC). It is not well evaluated in terms of ACS prevention. Here we report a 23-year-old patient with SCD and recurrent ACS; she was started on Crizanlizumab and she had no more ACS, but once she was off Crizanlizumab she developed ACS again, later Crizanlizumab was re-started, and the patient has improved significantly.

Hemoglobin D-Los Angeles is a variant of hemoglobin that can polymerize in the deoxygenated state. When co-inherited with Hemoglobin S (HbSD-Los Angeles disease) a severe sickling syndrome similar to HbSS can result. Corona virus infectious disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome-corona virus-2. It has been associated with acute chest syndrome (ACS) in individuals with sickle cell disease (SCD), but this complication has not previously been reported in patients with HbSD-Los Angeles. Dexamethasone has been shown to improve outcomes in non-SCD patients with severe acute respiratory syndrome-corona virus-2 pneumonia or acute respiratory distress syndrome; however, its use in SCD patients with ACS is controversial due to a reported increased risk of complications including vaso-occlusive painful episodes. Herein, we reported a patient with HbSD-Los Angeles and COVID-19-associated ACS whom we treated with dexamethasone without transfusion. The patient experienced a rapid recovery without sequelae from steroid use. To further evaluate the use of steroids, we conducted a literature review focusing on the management of pediatric SCD patients with COVID-19-associated ACS. We identified a total of 39 pediatric patients with SCD and COVID-19, of whom 21 (54%) had ACS. Packed red blood cell transfusion (n=11), exchange transfusion (n=4), or a combination of exchange transfusion and packed red blood cell transfusion (n=4) were the most frequently reported treatment, with hydroxychloroquine (n=5), remdesivir (n=1), and tocilizumab (n=1) also being reported. Three patients were treated with dexamethasone. All patients recovered and no adverse outcomes from steroid use were reported. Even though transfusion is considered the standard of care for children with ACS and steroids are not routinely recommended, our experience suggested that COVID-19-associated ACS may be an important exception, especially for patients who refuse transfusion or are in resource-poor nations where blood transfusions may not be readily available. Further studies are warranted to confirm these observations.

Acute chest syndrome (ACS) is a common cause of death in sickle cell disease (SCD) patients. Multiple studies investigated the risk factors of developing ACS; however, predictors of recurrent ACS episodes have not been thoroughly investigated. We aim to examine the clinical and laboratory predictors of recurrent ACS in pediatric patients with SCD. A retrospective case-control study included pediatric patients with SCD (˂14 years) admitted with ACS or developed ACS during admission for another indication. Patients were classified into recurrent ACS episodes (≥2 episodes) and a single ACS episode groups. Ninety-one ACS episodes (42 patients) were included, with a mean age at diagnosis of 7.18 ± 3.38 years. Twenty-two (52.4%) patients were male, and twenty-five (59.5%) patients had recurrent ACS. Younger age at first ACS was significantly associated with recurrence (p = 0.003), with an optimal cutoff at 7.5 years (area under the receiver operating characteristic curve [AUROC] = 0.833; p < 0.001). Higher SCD-related hospitalizations were significantly associated with recurrence (p = 0.038). Higher mean values of baseline white blood count (WBC) (p = 0.009), mean corpuscular volume (MCV) (p = 0.011), and reticulocyte (p = 0.036) were significantly associated with recurrence. Contrarily, lower baseline hematocrit values (p = 0.016) were significantly associated with recurrence. The ACS frequencies were significantly lower after hydroxyurea (p = 0.021). The odds of ACS recurrence increased with a positive C-reactive protein (CRP) at admission (p = 0.006). In conclusion, several baseline and admission laboratory data showed significant associations with recurrence. Hydroxyurea therapy demonstrated reduced ACS episodes.

This is a case report about a 7-year-old male child with sickle cell anemia (S/β+) who died unexpectedly during hospitalization, justifying the performance of a forensic autopsy completed by histological examination of organ fragments and toxicological analyses of biological fluids. The diagnosis retained was pulmonary thromboembolism as the cause of death occurring in the context of an acute chest syndrome (ACS). The mechanism of occurrence of this pulmonary embolism was vascular stasis caused by sickle cell disease. The search for etiologies of ACS complicating sickle cell disease should not exclude pulmonary embolism with red cell dense fibrin clot..

Acute chest syndrome (ACS) is a severe form of vaso-occlusive crisis, which is a main feature of sickle cell disease (SCD), an inherited hemoglobinopathy. Traditionally, hydroxyurea has been the treatment of choice for SCD to prevent vaso-occlusive crises including ACS. However, hydroxyurea may be contraindicated, for example, in patients wanting to have children. We here present a young male with SCD who wanted to become a father and developed a life-threatening episode of ACS following discontinuation of hydroxyurea and switching to partial exchange blood transfusions. The patient, aged 32 years and originally from Bahrain, had been diagnosed with homozygous SCD, alpha-thalassemia, and glucose-6-phosphate dehydrogenase deficiency as a child. He had an episode of ACS with moderate severity in 2008, after which he started using hydroxyurea. From 2008 until the present, he did not experience any episodes of ACS. About six months before the present episode, he stopped using hydroxyurea and switched to partial exchange transfusions, aiming to keep hemoglobin S (HbS) below 30%. The interval between the transfusions was typically about seven to eight weeks. On the evening (day 1) before hospital admission, he developed typical symptoms and signs of vaso-occlusive crisis, and during the first day in the hospital (HbS about 55%), his pulmonary function deteriorated, and he also developed cerebral symptoms (somnolence and confusion). On suspicion of ACS, a full blood exchange transfusion was administered on day 3. He then gradually recovered clinically, and his laboratory values also normalized. He was discharged on day 10. Subsequent follow-up visits at the outpatient clinic the following month were unremarkable. Possibly, this severe episode of ACS was triggered by switching from hydroxyurea therapy to partial exchange transfusions with too long intervals between the transfusions. This novel case is a compelling reminder of the possible perils that may accompany the discontinuation of hydroxyurea, the best-documented therapy in SCD.