Sickle Cell Disease Diagnosis and Management

Clinical Manifestations

The presence of sickle cells in the hemogram is diagnostic of sickle cell disease, which can present in different genotypes (HbSS, HbSC, HbS/β-thalassemia), according to the American Academy of Pediatrics 1
Patients with sickle cells in the hemogram may experience increased susceptibility to infections due to splenic dysfunction, which can begin as early as 3 months of age, as reported by the American Academy of Pediatrics 1
Sickle cell disease patients may present with acute chest syndrome, characterized by segmental pulmonary infiltrate, respiratory symptoms, chest pain, and/or hypoxemia, as described by the American Academy of Pediatrics 2
Secuestro esplénico agudo, which can progress rapidly to shock and death, is a complication of sickle cell disease, according to the American Academy of Pediatrics 2
Transient aplastic crisis, characterized by exacerbation of baseline anemia with decreased reticulocyte count, is a complication of sickle cell disease, as reported by the American Academy of Pediatrics 2
Stroke, which requires urgent evaluation in the presence of any acute neurological symptoms, is a complication of sickle cell disease, according to the American Academy of Pediatrics 2
Priapism, which can occur in children and adolescents, is a complication of sickle cell disease, as described by the American Academy of Pediatrics 2

Anemia is a common finding in patients with sickle cell disease, with a normocytic normochromic anemia pattern, as reported in Kidney International Supplements 3
Elevated reticulocyte count is a response to hemolysis in sickle cell disease patients, according to The Journal of Urology 4
In acute vaso-occlusive crises, patients may experience leucocytosis, thrombocytosis, and increased mean platelet volume, as described in The Journal of Urology 4

Newborn screening programs can detect sickle cell disease, where available, as recommended by the American Academy of Pediatrics 1
Hemoglobin electrophoresis can confirm the presence of hemoglobin S, according to the American Academy of Pediatrics 1

Pain management with analgesics, adequate hydration, and, in severe cases, parenteral opioids is a treatment approach for sickle cell disease, as described by the American Academy of Pediatrics 2