People with sickle cell disease experience a high incidence of chronic kidney disease and end-stage kidney disease, secondary to tubular and glomerular effects of vaso-occlusion-induced hypoxia. Because of concerns of suboptimal kidney function, sickle cell donors are usually not considered for kidney donation, even if the rest of the parameters are acceptable for organ donation. A significant gap exists between the number of organ donors and the number of candidates waiting for a kidney transplant in the United States. To bridge the gap, we need to consider using nontraditional donors. We report kidney transplant outcomes in 6 recipients from 4 sickle cell kidney donors. Intracranial hemorrhage and sepsis were the causes of the death in donors, and no donor was in sickle cell crisis at the time of donation. None of the recipients experienced delayed graft function, and all recipients achieved excellent allograft function. The earliest allograft failure was at 27 months in a recipient who developed early acute rejection, while the longest follow-up was 10 years with adequate kidney function. In conclusion, given the shortage of kidneys for transplantation and demonstrated good outcomes, we propose that kidneys from sickle cell donors can be safely used.

UNASSIGNED: Describe the various presentations of the rare entity of orbital wall infarction secondary to sickle cell disease and highlight the importance of magnetic resonance imaging in differentiating the entity from other similar diagnoses.
UNASSIGNED: A 4-year-old child presented to the hospital with bilateral recurrent painless orbital wall infarction secondary to sickle cell disease. Orbital wall infarctions have been described before in the literature; However, the painless and recurrent nature is intriguing.
UNASSIGNED: Orbital wall infarctions secondary to sickle cell disease represent an unusual presentation of the disease and often pose a diagnostic dilemma. When considering differentiating orbital wall infarctions from other resembling entities, magnetic resonance imaging is considered superior to computed tomography due to its ability to delineate the ischemic changes in the bone marrow, which further aids in the diagnosis. In situations where the orbital wall infarction does not lead to orbital compression syndrome, a conservative approach should suffice.

Choledochal cyst is a congenital pathology with an uncommon anomaly associated with common complaints of an abdominal lump and hepatic dysfunction. It may be presented equally in any phase of life, be it childhood, adolescence, or adulthood, and is majorly detected by ultrasonography (USG) on the appearance of primary symptoms in the hepato-biliary system. It has a classical triad consisting of a lump in the upper quadrant on the right side of the abdomen, pain in the upper part of the abdomen, and obstructive jaundice. A few of the clinical features overlap with sickle cell disease. A 30-year-old male patient with sickle cell anemia was diagnosed eight years ago. The patient was diagnosed with a choledochal cyst with the clinical presentation of abdominal pain, nausea, and vomiting, which hampered his routine life. Due to symptomatic recurrence, the patient was subjected to USG (abdomen), which showed a dilated common bile duct (CBD) and dilated intrahepatic biliary radicals. This is a rare case presentation with both sickle cell disease and choledochal cyst, which are symptomatically similar. Based on history, risk factor analysis, and diagnostic findings, the patient was advised to have a Roux-en-Y hepatico-jejunostomy. Endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP) are the investigations of choice, with the better being MRCP. ERCP is a therapeutic and diagnostic modality that helps in the removal of CBD calculus and the placement of a stent. There may be increased bilirubin, showing features of obstructive jaundice in alcoholic stools. In surgical management, which is of total excision of the cyst, there are vital structures in proximity. The patients with these complaints need to be evaluated thoroughly, and detailed clinical examination and proper radiological investigations will be performed. Roux-en-Y hepatico-jejunostomy with cyst excision in toto is the procedure of choice.

UNASSIGNED: Left ventricular systolic dysfunction (LVSD) is an uncommon but life-threatening complication of sickle cell disease (SCD), with poorly characterized aetiology. We present three SCD patients with LVSD due to different underlying mechanisms.
UNASSIGNED: The first case describes rapid deterioration in LV function secondary to severe cardiac iron overload in a 37-year-old female with poor chelation compliance after 10 years of top-up transfusions for SCD. The second case is a severe non-ischaemic dilated cardiomyopathy (DCM) in a 42-year-old SCD patient with longstanding sickle nephropathy and hypertension. The final case demonstrates severe LVSD with large transmural infarcts (ischaemic DCM) in the absence of epicardial coronary disease in a 52-year-old SCD patient.
UNASSIGNED: This case series presents the first attempt to characterize the aetiology of LVSD in SCD. We identified three phenotypes: iron-overload cardiomyopathy, non-ischaemic DCM, and ischaemic DCM. These contrasting cases highlight the significance of understanding the underlying pathology in determining individualized treatment plans for these high-risk patients. We discuss the role of cardiac MRI (CMR) in characterizing LV dysfunction, and we believe that this case series will form the basis of prospective studies to further delineate the pathophysiology of LVSD in SCD.

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.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially life-threatening syndrome characterized by excessive immune activation and tissue inflammation. This case report describes the early diagnosis of HLH in an adult patient who initially presented with a febrile syndrome associated with low back pain. The patient, a 33-year-old male, exhibited bicytopenia, hepatosplenomegaly, and hyperferritinemia without a previous diagnosis of sickle cell disease (SCD). Diagnostic challenges arose due to the overlapping clinical manifestations of SCD and HLH and their uncommon association. However, timely recognition and intervention were achieved through comprehensive diagnostic evaluations, including a bone marrow biopsy. The patient was promptly started on an appropriate therapeutic regimen, which led to significant clinical improvement. This case underscores the importance of considering HLH in the differential diagnosis of adults presenting with hematologic abnormalities and systemic inflammation. Early diagnosis and treatment are critical to improving outcomes for patients with this complex and severe disorder.

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.

Sickle cell disease (SCD) is an autosomal recessive genetic disorder characterized by the abnormal formation of sickle hemoglobin (HbS). Under conditions of deoxygenation, HbS undergoes polymerization, resulting in microvascular occlusion, tissue hypoxia, and infarction. The elevated mortality rate associated with SCD is primarily attributed to complications such as sepsis, acute chest syndrome, stroke, acute multiorgan failure, and pulmonary hypertension. Despite advancements in awareness and treatments, preventing mortality in young individuals with SCD remains a formidable challenge. In an effort to shed light on these challenges, we present a case of unexpected death associated with SCD to emphasize the pressing need for continued research and intervention strategies to improve patient outcomes.

OBJECTIVE: To corroborate the vascular etiology of sudden sensorineural hearing loss (SNHL) utilizing magnetic resonance imaging (MRI).
METHODS: A 24-year-old male with a history of sickle cell disease experienced sudden SNHL and right horizontal nystagmus, without accompanying vertigo.
METHODS: Audiometric evaluation revealed left-sided SNHL, predominantly affecting high frequencies. Video head impulse testing demonstrated isolated dysfunction of the left posterior semicircular canal. An urgent brain MRI identified a recent punctiform ischemic stroke in the frontal region. A subsequent MRI, conducted with a 4-hour delay and post-contrast enhancement, highlighted a hyperintense signal within the left cochlear region and the left posterior semicircular canal.
CONCLUSIONS: The investigative results substantiate an infarction in the territory of the cochlear artery, precipitated by a vaso-occlusive event, thereby reinforcing the vascular hypothesis of cochleovestibular artery syndrome. This case underscores the congruence between clinical observations and delayed post-contrast MRI findings.

Sickle cell disease (SCD) and systemic lupus erythematosus (SLE) are two uncommon disorders each characterized by multisystemic manifestations. Individuals with SCD exhibit abnormalities in the complement pathway, which may predispose patients to develop autoimmune disorders such as SLE. As many manifestations of SLE mimic those of SCD, diagnosis and therapeutic management of SLE in a patient with known SCD may be delayed. In this study, we describe our institutional experience of diagnosing and managing concomitant SCD and SLE. We offer insights into the complex interplay between these conditions to enhance early recognition and effective management of concurrent SCD and SLE.