Case Presentation:
A 27‐year‐old woman with sickle cell disease presented with acute bilateral leg, abdominal, and chest pain accompanied by nausea, nonbloody vomit, and a productive cough. The woman stated that the pain was consistent with her usual pain crisis for which she was hospitalized once yearly. The patient had never had a blood transfusion reaction or sickle cell disease complications including acute chest syndrome, stroke, renal failure, and myocardial infarction. The patient was oxygenating 92% on room air, was afebrile, had crackles at the left lung base, had a normal electrocardiogram, and had stable cardiomegaly on chest radiograph. Approximately 24 hours after initiation of supportive pain crisis treatment, the patient experienced increasing chest pain and shortness of breath with corollary changes on electrocardiogram. Repeat chest radiograph showed the interval development of a prominent infiltrate. The patient was transferred to the intensive care unit for exchange transfusion and close monitoring of presumed acute chest syndrome symptoms. Because of precipitously increasing oxygen requirements, elective intubation was initiated. In the midst of intubation, the patient's systolic blood pressure dropped from 150 to pulseless electrical activity. Despite aggressive recuscitation, the patient died. Autopsy showed hepatomegaly with diffuse microsteatosis and cardiomegaly. Death due to hepatic sequestration/reverse sequestration was declared.
Discussion:
Sickle cell disease pain crisis has many potential complications. Although trained to quickly recognize acute chest syndrome, stroke, and priapism, providers must also consider rarer, less easily recognized complications, even when symptoms are consistent with uncomplicated pain crisis. Hepatobiliary complications of sickle cell disease include acute sickle hepatic crisis, sickle cell intrahepatic cholestasis, benign hyperbilirubinemia, hepatic infarction, and Budd‐Chiari syndrome. Hepatic sequestration and its rare progression to reverse sequestration may also occur. Patients with hepatic sequestration usually present with nonspecific right upper quadrant pain and hepatomegaly. Pathophysiologically, hepatic sequestration results from red blood cells becoming sequestered in the liver. Acute anemia, hepatomegaly, shock, and sometimes death ensue. Hepatic sequestration can be followed by reverse sequestration in which the viable sequestered cells are presumed to be released back into the circulation causing a spontaneous and rapid rise in hemoglobin. Hypervolemia, hypertension, heart failure, and intracerebral hemorrhage results. Treatment consists of early identification and intervention with exchange transfusion.
Conclusions:
Because markers are nonspecific and sequelae are rapid, early diagnosis and treatment are challenging. Careful monitoring of blood pressure, liver function tests (particularly for Tbili > 13), blood counts, hemoglobin, hemoglobin S, and plasma viscosity is recommended.
Author Disclosure:
L. Wasson, none; J. Bhutto, none.