Successful Outcome of Chronic Intrahepatic Cholestasis in an Adult Patient with Sickle Cell/β+Thalassemia

Vlachaki, Efthymia; Andréadis, P; Neokleous, Nikolaos; Agapidou, Aleka; Vetsiou, Evaggelia; Katsinelos, Panagiotis; Boura, Panagiota

doi:10.1155/2014/213631

Cited by 6 publications

(10 citation statements)

References 9 publications

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“…Other means of SCD management must also be considered such as routine follow-up with a hematologist. Hydroxyurea therapy has been well-established in SCD, and may be effective in preventing acute exacerbations of SCIC [12]. Another consideration may be allogeneic stem cell transplant (SCT), the only currently available Pdf_Folio:130 definitive cure for SCD.…”

Section: Discussionmentioning

confidence: 99%

“…Few cases have been previously described. Patients present with chronically elevated bilirubin, as is characteristic of SCD, likely due to baseline hemolysis as well as liver dysfunction [5,[10][11][12][13][14]. We herein present a case report of a patient we saw in routine practice and followed thereafter.…”

Section: Presentation Of Chronic Scic With Acute Exacerbationmentioning

confidence: 99%

“…SCIC can present acutely or recurrently as chronic process which may give rise to overt liver failure [5,7,9]. To date, seven cases of chronic SCIC have been described in adults, and the entity has no fixed clinical or histologic criteria [5,[10][11][12][13][14]. Symptomology is defined by pronounced hyperbilirubinemia with variable associated symptoms and intermittent exacerbating episodes of acute SCIC [5,7,9].…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Presentation Of Chronic Scic With Acute Exacerbationmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Adkins¹,

Savani²,

Booth³

2019

CHI

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“…Recent case reports have confi rmed the successful outcome with ET. Eight adult cases (5,(11)(12)(13)(14)(15)(16) (Table 2). Seven out of the eight patients received ET, and two of them progressed to hepatic failure and death (5,13).…”

Section: Discussionmentioning

confidence: 99%

“…Seven out of the eight patients received ET, and two of them progressed to hepatic failure and death (5,13). Two of the patients continued to receive regular ET to maintain their hemoglobin S level (14,16). Overall, there are 24 case reports in the adult population.…”

Section: Discussionmentioning

confidence: 99%

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

Hosiriluck¹,

Rassameehiran²,

Argueta³

et al. 2014

Baylor University Medical Center Proceedings

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Sickle Hepatopathy

Praharaj

Anand

2021

Journal of Clinical and Experimental Hepatology

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Sickle hepatopathy is an umbrella term describing various pattern of liver injury seen in patients with sickle cell disease. The disease is not uncommon in India; in terms of prevalence, India is second only to Sub-Saharan Africa where sickle cell disease is most prevalent. Hepatic involvement in sickle cell disease is not uncommon. Liver disease may result from viral hepatitis and iron overload due to multiple transfusions of blood products or due to disease activity causing varying changes in vasculature. The clinical spectrum of disease ranges from ischemic injury due to sickling of red blood cells in hepatic sinusoids, pigment gall stones, and acute/chronic sequestration syndromes. The sequestration syndromes are usually episodic and self-limiting requiring conservative management such as antibiotics and intravenous fluids or packed red cell transfusions. However, rarely these episodes may present with coagulopathy and encephalopathy like acute liver failure, which are life-threatening, requiring exchange transfusions or even liver transplantation. However, evidence for their benefits, optimal indications, and threshold to start exchange transfusion is limited. Similarly, there is paucity of the literature regarding the end point of exchange transfusion in this scenario. Liver transplantation may also be beneficial in end-stage liver disease. Hydroxyurea, the antitumor agent, which is popularly used to prevent life-threatening complications such as acute chest syndrome or stroke in these patients, has been used only sparingly in hepatic sequestrations. The purpose of this review is to provide insights into epidemiology of sickle cell disease in India and pathogenesis and classification of hepatobiliary involvement in sickle cell disease. Finally, various management options including exchange transfusion, liver transplantation, and hydroxyurea in hepatic sequestration syndromes will be discussed in brief. (

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Successful Outcome of Chronic Intrahepatic Cholestasis in an Adult Patient with Sickle Cell/β⁺Thalassemia

Cited by 6 publications

References 9 publications

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

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

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

Sickle Hepatopathy

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