Acute Sickle Hepatic Crisis After Liver Transplantation in a Patient With Sickle β-Thalassemia

Perini, Guilherme Fleury; Santos, Fabio P S; Neto, José Ben Hur Ferraz; Pasqualin, Denise da Cunha; Hamerschlak, Nelson

doi:10.1097/tp.0b013e3181e8a6b3

Cited by 15 publications

(13 citation statements)

References 1 publication

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“…Hepatic crisis is often used as a general term to describe right upper quadrant pain in a sickle cell patient [80, 81]. However, hepatic crisis is best used to describe a syndrome consisting of pain, elevated ALT (usually less than 300 IU/liter), and hepatic enlargement.…”

Section: Gastrointestinal/hepatobiliary Complicationsmentioning

confidence: 99%

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Ballas

Kesen

Goldberg

et al. 2012

The Scientific World Journal

153

144

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The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

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Section: Gastrointestinal/hepatobiliary Complicationsmentioning

confidence: 99%

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Ballas

Kesen

Goldberg

et al. 2012

The Scientific World Journal

153

144

View full text Add to dashboard Cite

show abstract

“…1,17,18,[27][28][29][30][31][32][33][34][35][36][37] However, several questions remain unanswered. The poor results reported in early case series have improved, to some degree, in later studies.…”

Section: What Is the Role Of Lt?mentioning

confidence: 99%

How we treat sickle hepatopathy and liver transplantation in adults

Gardner

Suddle²,

Kane

et al. 2014

Blood

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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.

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“…A review of the literature identified 10 adult and 5 children with sickle cell disease (Table 1). 2,5,6,9-17 In 6 of the 10 reported adult cases, liver transplantation resulted in improved function and long-term survival. Of the 4 that resulted in post-OLT death, 2 were from causes unrelated to the OLT or liver dysfunction (1 from intracranial bleed 5 months post-op due to pre-existing vascular anomaly and 1 from pulmonary embolism 22 months post-op).…”

Section: Discussionmentioning

confidence: 99%

Successful Orthotopic Liver Transplantation in an Adult Patient with Sickle Cell Disease and Review of the Literature

et al. 2013

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Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and underwent successful orthotopic liver transplantation. Both pre and post-operatively, he was maintained on red cell transfusions. He remains stable with improved liver function 42 months post transplant. The role for orthotopic liver transplantation is not well defined in patients with sickle cell disease, and the experience remains limited. Although considerable challenges of post-transplant graft complications remain, orthotopic liver transplantation should be considered as a treatment option for sickle cell disease patients with end-stage liver disease who have progressed despite conventional medical therapy. An extended period of red cell transfusion support may lessen the post-operative complications.

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Acute Sickle Hepatic Crisis After Liver Transplantation in a Patient With Sickle β-Thalassemia

Cited by 15 publications

References 1 publication

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

How we treat sickle hepatopathy and liver transplantation in adults

Successful Orthotopic Liver Transplantation in an Adult Patient with Sickle Cell Disease and Review of the Literature

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