Fulminant liver failure in a 12-year-old girl with sickle cell anaemia: favourable outcome after exchange transfusions

Abstract

Massive hepatic sickling should be considered in the differential diagnosis of a child with homozygous sickle cell disease who suddenly develops acute liver failure. Exchange transfusion should be promptly carried out so as to reverse ischaemic hepatic injury.

Sickle cell hepatopathy

“…Hypofibrinogenemia, thrombocytopenia, and lactic acidosis may accompany the liver failure. 14 A Tc-99m sulfur colloid liver spleen scan done in a single patient showed hepatomegaly with patchy uptake of colloid. 15 Postmortem liver biopsies in 4 patients with sickle intrahepatic cholestasis showed dilated canaliculi with bile plugs.…”

“…The characteristic finding is that of strikingly high plasma bilirubin concentrations, with a level of 273 mg/dL documented in 1 patient. 14 In most cases the conjugated fraction exceeds 50% of the total bilirubin. The extremely high bilirubin levels are caused by a combination of on-going hemolysis, intrahepatic cholestasis, and renal impairment.…”

“…7,16,18 Sixteen probable cases have been reported to date, 7,14-22 and whereas the syndrome proved fatal in 9 early cases, recent reports have described reversal of this process within 48 hours in 7 patients, with vigorous exchange transfusions and correction of coagulopathy with fresh frozen plasma. [14][15][16][20][21][22] The renal impairment in sickle intrahepatic cholestasis has not been well studied, but appears reversible and improves concurrently with hepatic improvement. 16,21 Urine electrolytes were measured in 1 case and suggested acute tubular necrosis, 21 but the investigators attributed this to aminoglycoside therapy administered to the patient.…”

Acute liver failure in a patient with sickle cell/β+ thalassaemia

“…Although initial reports have suggested a universally poor prognosis, with death occurring from bleeding and liver failure, successful outcomes have been described using intensive supportive care and exchange transfusion. [7][8][9]…”

Is there a role of exchange transfusions in patients with sickle cell anemia and major liver surgery?

“…'Hepatic crisis' occurs in 10% of patients and are associated with few signs and symptoms. Even though the liver is protected by its dual blood supply, sinusoidal obstruction by enlarged Kuppfer cells and clumps of sickle red cells causing degenerative liver lesions have been reported [3]. Hepatic focal necrosis, portal fibrosis, regenerative nodules and cirrhosis is a wide range of pathologic events described in an autopsy series of SC patients from Johns Hopkin's [4].…”