Reversal of Liver Function Without Exchange Transfusion in Sickle Cell Intrahepatic Cholestasis

Hosiriluck, Nattamol; Rassameehiran, Supannee; Argueta, Erwin; Tijani, Lukman

doi:10.1080/08998280.2014.11929160

Cited by 6 publications

(6 citation statements)

References 14 publications

(21 reference statements)

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“…There are no randomized control trials to date assessing the optimum treatment for this condition [ 8 ]. Three cases have been reported in which SCIC was managed without exchange transfusion [ 9 ]. Hydroxyurea increases levels of HbF and is often used to prevent complications in sickle cell patients, including painful crisis, acute chest syndrome, priapism, and stroke, and decrease the need for blood transfusion.…”

Section: Discussionmentioning

confidence: 99%

Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies

et al. 2015

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Patient: Male, 31Final Diagnosis: Sickle cell intrahepatic cholestasisSymptoms: Abdominal pain • fever • jaundiceMedication: —Clinical Procedure: Exchange transfusionSpecialty: HematologyObjective:Rare diseaseBackground:Sickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality.Case Report:We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice. There was an accelerated rise in total bilirubin to 50 mg/dL, direct bilirubin 38 mg/dL, and Cr 3.0 mg/dL. Hb was 6.4 g/dL, reticulocyte count 16%, ALT 40 IU/L, AST 155 IU/L, ALP 320 IU/L, and LDH 475 IU/L. Hepatitis panel was negative and MRCP showed normal caliber of the common bile duct, with no obstruction. Exchange transfusion of 9 units of packed red blood cells led to great improvement in his condition.Conclusions:SCIC, unlike the other sickle cell hepatopathies, requires urgent and vigorous exchange transfusion. Renal impairment in SCIC has not been well studied but usually is reversible with the hepatic impairment, as in this case. Unresolved renal impairment requires dialysis and is associated with poor outcome. There is limited data on use of hydroxyurea to prevent SCIC, and liver transplant is associated with high mortality. A timely diagnosis of SCIC and appropriate management is life-saving.

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Section: Discussionmentioning

confidence: 99%

Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies

et al. 2015

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“…Exchange transfusion (ET) is the only effective treatment for SCIC; early intervention with exchange transfusion helps to minimize sickling thus lowering the fraction Hemoglobin S (ideally below 30%). [2] ET is the only intervention which decreases patient mortality as compared to solely supportive measures. [2] Cholelithiasis and viral hepatitis must be ruled out before a diagnosis of SCIC can be made (often a diagnosis of exclusion).…”

Section: Introductionmentioning

confidence: 99%

“…[2] ET is the only intervention which decreases patient mortality as compared to solely supportive measures. [2] Cholelithiasis and viral hepatitis must be ruled out before a diagnosis of SCIC can be made (often a diagnosis of exclusion). [4] The disease can present in its most severe form when the patient is already coagulopathic with renal failure and ultimately fulminant hepatic failure.…”

Section: Introductionmentioning

confidence: 99%

Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion

Likhtshteyn

Iqbal

McFarlane

et al. 2020

AJMCR

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With the advent of hydroxyurea, the sickle cell population has been enjoying a prolonged life span as compared to the pre-hydroxyurea era. Traditionally, acute complications of sickle cell disease includes acute chest syndrome, MI and stroke. In this report we present a case of an elderly man with sickle cell disease who presented with intrahepatic cholestasis (SCIC); a rather rare and fatal complication of sickle cell hemoglobinopathy. The patient presented with jaundice and elevated bilirubin up to 53, his hospital course was complicated by coagulopathy and encephalopathy, and expired on day 43 of presentation after failing multiple therapeutic interventions including exchange transfusion. In this report, we will provide literature review and discuss the underlying pathophysiologic mechanisms of intrahepatic cholestasis in the sickle cell population highlighting the need for immediate recognition and institution of therapy for this fatal complication of sickle cell disease, particularly in elderly populations with low metabolic reserve.

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“…In the intervening years, significant advances have been made in the management of SCD, and supportive management utilized in the treatment of SCIC has progressed with improvements in dialysis, Source: Data compiled and presented with permission from Costa et al [27], Hosiriluck et al [22], Im et al [38], and Guimarães et al [28].…”

Section: Introductionmentioning

confidence: 99%

“…Reported cases of SCIC have been routinely managed with red blood cell exchange transfusion (RBCEx). Of cases reported since 1980, two patients survived without the use of RBCEx; however, due to the rarity and risk of mortality, a direct comparison through randomized controlled trials (RCT) of simple transfusion versus RBCEx has not been performed [21][22][23][24].…”

Section: Introductionmentioning

confidence: 99%

Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion

Adkins¹,

Savani²,

Booth³

2019

CHI

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Sickle cell disease patients are commonly treated at transfusion medicine services, and understanding of the hepatic manifestations of the disease is key for optimal management, specifically, in individuals presenting with sickle cell intrahepatic cholestasis (SCIC). SCIC represents a rare, severe hepatic crisis wherein sinusoidal red cell sickling leads to massive hepatocyte dysfunction and cholestatic laboratory findings. Acute SCIC is defined by abdominal pain with progressive hepatic injury associated with hyperbilirubinemia, renal failure, encephalopathy, and coagulopathy. Patients are generally managed with red blood cell exchange transfusion (RBCEx), when available, as this is a potentially fatal condition. Simple transfusion may be utilized in resource-poor environment or when patients refuse RBCEx. As less than 50 adult cases have been described in the literature, many of them with limited follow-up, randomized clinical trials comparing RBCEx with other treatments are currently unfeasible. Likewise, a chronic form exists, but is less well characterized, and is associated with persistent bilirubinemia and a variable course in terms of progressive hepatic disease. We undertake a brief review of the literature and discuss two cases of SCIC managed with RBCEx at our institution.

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Reversal of Liver Function Without Exchange Transfusion in Sickle Cell Intrahepatic Cholestasis

Cited by 6 publications

References 14 publications

Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies

Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies

Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion

Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion

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