Acute Sickle Hepatic Crisis After Liver Transplantation in a Patient With Hb SC Disease

Abstract

Acute sickle hepatic crisis (ASHC) has been observed in approximately 10% of patients with sickle cell disease. It occurs predominantly in patients with homozygous (Hb SS) sickle cell anemia and to a lesser degree in patients with Hb SC disease, sickle cell trait, and Hb S beta thalassemia. Patients commonly present with jaundice, right upper quadrant pain, nausea, low-grade fever, tender hepatomegaly, and mild to moderate elevations in serum AST, ALT, and bilirubin. We describe the case of a patient with a history of hemoglobin SC disease and cirrhosis caused by hepatitis C presenting approximately 1 year after liver transplantation with an ASHC. The diagnosis was confirmed by liver biopsy. Our patient was treated with RBC exchange transfusions, IV hydration, and analgesia and made a complete recovery. Only a limited number of patients with sickle cell disease have received liver transplants, and, to our knowledge, this is the first case of ASHC after transplantation in a patient with Hb SC disease.

Liver transplantation in patients with sickle cell disease: possible but challenging—a cohort study

“…In practice, LT can be indicated in two settings [4]: acute liver failure mimicking fulminant hepatitis and end‐stage chronic liver disease. Data regarding liver transplantation (LT) in patients with SCD are limited to mostly case reports (Table 1) [5‐24]. Because of the paucity of data available regarding the indications for LT and perioperative outcomes as well as the lack of long‐term follow‐up in most reported cases, the present study entailed a thorough analysis of a large single‐center experience of LT in patients with SCD.…”

Indicações e contra-indicações no transplante hepático pediátrico

“…Outro aspecto particular é o acometimento da criança em um período crítico de crescimento e desenvolvimento, do que decorre o caráter mais urgente do transplante pediátrico, para que possíveis déficits possam ser evitados ou revertidos. Mais recentemente, o diagnóstico precoce de algumas doenças metabólicas, com ou sem comprometimento neurológico, também são indicação de transplante 1 .…”