Acute Hepatic Sequestration in Sickle Cell Disease: DISCUSSION
Based on the presentation and work-up, it is likely that this patient presented with acute hepatic sequestration. Acute hepatic sequestration is characterized by right upper quadrant pain and hepatomegaly. The laboratory data show hyperbilirubinemia, low hemoglo-bin/hematocrit with reticulocytosis. The transaminases are minimally elevated, and the CT and abdominal ultrasound show diffuse hepatomegaly. Viral hepatitis was considered an unlikely culprit. It rarely causes an extensive hyperbilirubinemia, and the transaminases remain elevated for a greater time period.
Patients with sickle cell disease are prone to biliary tract disease. The lack of sonographic evidence of stones, a serum bilirubin level greater than 30 mg/ dl in the presence of normal renal function, led us away from the diagnosis of biliary tract disease. The diagnosis of right upper quadrant hepatic syndrome was excluded. In adults, the clinical course is typically three-to-seven days. The serum bilirubin levels seldom exceed 15 mg/dl, and a low hematocrit has not been described in this syndrome. Furthermore, the patient’s clinical and biological profile improved rapidly after receiving the partial exchange transfusion. Sickle cell intrahepatic cholestasis has a presentation similar to acute hepatic sequestration. The diagnosis of intrahepatic cholestasis was excluded based on the physical examination and laboratory data. The biochemical markers show renal failure, thrombocytopenia, and a coagulopathic state. Patients with intrahepatic cholestasis may also have an accompanying encephalopathic state.
E. coli bacteremia was also excluded as a possible cause of the patient’s hepatic failure. The patient’s physical examination, laboratory values, and clinical course also led us away from this diagnosis. Patients with hepatic dysfunction due to E. coli bacteremia present with clinical indicators of septic shock. These include hypotension, encephalopathy, oliguria, renal dysfunction, metabolic acidosis, and thrombocytopenia. The transaminases are elevated with an accompanying coagulopathy. The patient did not have signs of multiorgan dysfunction. The role of the E. coli bacteremia in this patient’s sickle cell crisis is unclear. Cases have shown that viral and bacterial infections may play a role as a precipitating factor in vaso-occlusive crises. The patient had a negative urine culture and showed no clinical signs of a secondary infection, such as an abscess or skin lesion that could have been seeded. E. coli may be found in the biliary tree, but it is of no consequence unless an obstruction is present. E. coli is endogenous to the gastrointestinal tract, and it is possible that due to the patient’s underlying sullen immune system, this may have precipitated the crisis.
Hepatic disease is common in sickle cell disease. The intrahepatic vaso-occlusive disorders seen in sickle cell disease encompass a variety of syndromes that are not often seen in a sickle cell crisis. The courses vary between a self-limiting syndrome that remits with intravenous hydration and analgesia to death from bleeding and liver failure. It can arise from a variety of insults to the liver, including ischemia, vascular occlusion, and sequestration. This case is important because acute hepatic sequestration in sickle cell disease is a manifestation of the disease that is unfamiliar to many clinicians. When it arises, it puts clinicians in a potential quandary. It is important to recognize the self-limiting hepatic right upper quadrant syndrome and the potentially lethal intrahepatic crisis and acute hepatic sequestration syndromes. Most clinicians are inclined to initially treat the more severe forms of this syndrome with simple blood transfusions, which may lead to hyperviscosity syndrome. A more prudent option is to perform a partial red blood cell exchange transfusion, prior to a red blood cell transfusion. The partial red blood cell exchange transfusion will reduce the number of sickled cells and increase the hemoglobin and hematocrit.
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