TABLE 1

Acute complications of sickle cell disease

Hepatic	Hepatic crisis: right upper quadrant pain, fever, jaundice, nausea, tender hepatomegaly, jaundice Hepatic sequestration: abdominal pain, tender hepatomegaly, and acute anemia, but absence of cholestasis or transaminitis Acute cholecystitis
Splenic sequestration	Sudden enlargement of spleen due to trapping of the red cell mass Presents with left-sided abdominal pain, abdominal distention, pallor, acute anemia, hypovolemic shock
Stroke	Focal seizures, hemiparesis, speech deficits; hemorrhagic stroke more common in adults
Acute ocular conditions	Hyphema, central retinal artery occlusion, orbital infarction, orbital compression syndrome
Acute chest syndrome	Fever, respiratory symptoms, chest pain, new infiltrate on chest radiography, hypoxia, acute anemia
Acute anemia	Decline of the hemoglobin level of 2 g/dL below the baseline value Etiology includes red cell aplasia, delayed hemolytic transfusion reaction, acute bleeding (surgery), spleen sequestration
Priapism	Painful sustained penile erection; urinary retention may occur
Fever	Repeated splenic infarctions from vaso-occlusion result in hyposplenism and functional asplenia, leading to increased susceptibility to infection from encapsulated organisms; sickle cell fever, defined as temperature > 38.3°C (101.5°F), should prompt rapid evaluation and initiation of antibiotics
Pain	Acute excruciating pain, most commonly in the extremities, chest, and back; onset may be gradual, duration may be hours to days; triggers include stress, exposure to cold, and infectious illness
Multisystem organ failure	Usually occurs during a vaso-occlusive crisis Presents with fever, rapid fall in hematocrit and platelet count, and altered sensorium; respiratory, hepatic, and kidney failure

Data from American Society of Hematology. Management of Acute Complications of Sickle Cell Disease: A Pocket Guide for the Clinician. www.hematology.org/Clinicians/Guidelines-Quality/Quick-Ref/3466.aspx.