How we treat sickle hepatopathy and liver transplantation in adults

TY - JOUR

T1 - How we treat sickle hepatopathy and liver transplantation in adults

AU - Gardner, Kate

AU - Suddle, Abid

AU - Kane, Pauline

AU - O'Grady, John

AU - Heaton, Nigel

AU - Bomford, Adrian

AU - Thein, Swee Lay

PY - 2014/4/10

Y1 - 2014/4/10

N2 - Sickle cell disease (SCD) has evolved into a debilitating disorder with emerging end-organ damage. One of the organs affected is the liver, causing "sickle hepatopathy," an umbrella term for a variety of acute and chronic pathologies. Prevalence of liver dysfunction in SCD is unknown, with estimates of 10%. Dominant etiologies include gallstones, hepatic sequestration, viral hepatitis, and sickle cell intrahepatic cholestasis (SCIC). In addition, causes of liver disease outside SCD must be identified and managed. SCIC is an uncommon, severe subtype, with outcome of its acute form having vastly improved with exchange blood transfusion (EBT). In its chronic form, there is limited evidence for EBT programs as a therapeutic option. Liver transplantation may have a role in a subset of patients with minimal SCD-related other organ damage. In the transplantation setting, EBT is important to maintain a low hemoglobin S fraction peri-and posttransplantation. Liver dysfunction in SCD is likely to escalate as life span increases and patients incur incremental transfusional iron overload. Future work must concentrate on not only investigating the underlying pathogenesis, but also identifying in whom and when to intervene with the 2 treatment modalities available: EBT and liver transplantation.

AB - Sickle cell disease (SCD) has evolved into a debilitating disorder with emerging end-organ damage. One of the organs affected is the liver, causing "sickle hepatopathy," an umbrella term for a variety of acute and chronic pathologies. Prevalence of liver dysfunction in SCD is unknown, with estimates of 10%. Dominant etiologies include gallstones, hepatic sequestration, viral hepatitis, and sickle cell intrahepatic cholestasis (SCIC). In addition, causes of liver disease outside SCD must be identified and managed. SCIC is an uncommon, severe subtype, with outcome of its acute form having vastly improved with exchange blood transfusion (EBT). In its chronic form, there is limited evidence for EBT programs as a therapeutic option. Liver transplantation may have a role in a subset of patients with minimal SCD-related other organ damage. In the transplantation setting, EBT is important to maintain a low hemoglobin S fraction peri-and posttransplantation. Liver dysfunction in SCD is likely to escalate as life span increases and patients incur incremental transfusional iron overload. Future work must concentrate on not only investigating the underlying pathogenesis, but also identifying in whom and when to intervene with the 2 treatment modalities available: EBT and liver transplantation.

KW - CELL INTRAHEPATIC CHOLESTASIS

KW - IRON OVERLOAD

KW - EXCHANGE TRANSFUSIONS

KW - DISEASE

KW - ANEMIA

KW - PATIENT

KW - CHILDREN

KW - FAILURE

KW - CHOLELITHIASIS

KW - THALASSEMIA

U2 - 10.1182/blood-2013-12-542076

DO - 10.1182/blood-2013-12-542076

M3 - Literature review

SN - 0006-4971

VL - 123

SP - 2302

EP - 2307

JO - Blood

JF - Blood

IS - 15

ER -