Acute complications of sickle cell disease

HepaticHepatic 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 sequestrationSudden enlargement of spleen due to trapping of the red cell mass
Presents with left-sided abdominal pain, abdominal distention, pallor, acute anemia, hypovolemic shock
StrokeFocal seizures, hemiparesis, speech deficits; hemorrhagic stroke more common in adults
Acute ocular conditionsHyphema, central retinal artery occlusion, orbital infarction, orbital compression syndrome
Acute chest syndromeFever, respiratory symptoms, chest pain, new infiltrate on chest radiography, hypoxia, acute anemia
Acute anemiaDecline 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
PriapismPainful sustained penile erection; urinary retention may occur
FeverRepeated 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
PainAcute 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 failureUsually occurs during a vaso-occlusive crisis
Presents with fever, rapid fall in hematocrit and platelet count, and altered sensorium; respiratory, hepatic, and kidney failure