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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 2  |  Page : 284-285
Sickle cell hepatopathy

1 Department of Pathology, Medical College, Kolkata 73, India
2 Department of Medicine, Nil Ratan Sircar Medical College, Kolkata-14, India

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Sickle cell hepatopathy is a well-documented entity that ranges from the self-limiting hepatic right upper quadrant syndrome to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. We describe a 26-year-male with homozygous sickle cell disease who had this unique hepatic presentation and was documented to have characteristic findings of cholestasis, portal inflammation and sinusoidal dilatation on histopathology.

Keywords: Histopathology, intrahepatic cholestasis, sickle cell hepatopathy

How to cite this article:
Bandyopadhyay R, Bandyopadhyay SK, Dutta A. Sickle cell hepatopathy. Indian J Pathol Microbiol 2008;51:284-5

How to cite this URL:
Bandyopadhyay R, Bandyopadhyay SK, Dutta A. Sickle cell hepatopathy. Indian J Pathol Microbiol [serial online] 2008 [cited 2021 Oct 18];51:284-5. Available from: https://www.ijpmonline.org/text.asp?2008/51/2/284/41698

   Introduction Top

Repeated episodes of intrahepatic sickling in a case of sickle cell disease results in recurrent attacks of abdominal pain with fluctuating intrahepatic cholestasis. [1] We report one such case in a 26-year-old male with homozygous sickle cell disease.

   Case History Top

A 26-year-male presented with persistent jaundice for last 12 years characterized by periods of fluctuation along with recurrent attacks of abdominal pain. Each abdominal attack used to last for few hours with localization mostly in the right hypochondrium. One such acute attack was so severe that the patient had to be admitted in our hospital. There was no symptom of weight loss, itching, altered mental status, involuntary movements, swelling of abdomen or feet, hematemesis, melena, bleeding, history of alcoholism, blood transfusion, surgery, or other major illness. He had multiple admissions earlier for pain abdomen that responded to symptomatic therapy. Personal and family history were non-contributory.

On examination, pallor, jaundice and clubbing were present. Firm non-tender hepatomegaly was noted with sharp margin and smooth surface. Rest of the examination was unrevealing.

A review of earlier investigations showed average hemoglobin between 9 and 11 g/dl, normocytic normochromic red cells and normal total, differential and platelets counts. Several reports of liver function tests revealed total bilirubin varying between 4 and 12 mg/dl, with conjugated fraction always more than 50% of total. Serum transaminases and alkaline phosphatase levels (with levels as high as 1200 IU/l) showed episodic fluctuations. Serum total protein, albumin and globulin were normal while the latest prothombin time value was 16.2 s (control 11 s). Abdominal ultrasound (done in 1999) revealed mild hepatomegaly with normal echopattern and hyperechoic portal triads. Upper gastrointestinal endoscopy was within normal limits. Serum HBsAg, HCV RNA, ANF, anti-LKM 1 , anti mitochondrial, anti-smooth muscle antibodies and HIV ELISA were non-reactive. Serum ferritin (387 g/l), ceruloplasmin (42 mg/dl) and copper (113 g/dl) were within normal limits. Liver biopsy (done in 2001) showed intralobular, portal and periportal inflammation, foci of hepatocyte degeneration, regeneration and piecemeal necrosis, no bile stasis/proliferation but presence of congested sinusoids.

To determine the cause of acute pain abdomen that required admission, we performed an urgent ultrasonography of abdomen that revealed hepatomegaly with normal echo pattern, normal biliary channels and a few small calculi in the gall bladder; the last one being a significant new finding. A significant reduction of hemoglobin (7.4 g%) was also noted when compared with earlier values. On the basis of these two new findings, a full blood count was advised that revealed Hb - 7.2 g/dl, hematocrit - 22%, MCV - 85.4 fL, MCH - 26.4 pg and a reticulocyte of 30%. A possibility of hemolytic anemia was strongly considered. Both direct and indirect Coomb's test was negative. Hb electrophoresis showed HbF - 13.1%, HbA - 1.5%, HbA2 - 3.6% and an HbS of 81.8%. Sickling test was positive. Detail study of family members revealed both the parents and the only sister to be heterozygous (with HbS levels of 40.7%, 32.8% and 35.6%, respectively) and the patient was diagnosed as homozygous sickle cell disease. Liver biopsy showed maintained basic architectural pattern with mild to moderate piecemeal necrosis, mild spotty necrosis, sinusoidal dilatation, mild to moderate inflammation and widening of the portal areas and cholestasis [Figure 1],[Figure 2],[Figure 3]. These findings together with the entire presentation were consistent with a final diagnosis of sickle cell hepatopathy.

   Discussion Top

Hyperbilirubinemia may punctuate the course of sickle cell anemia resulting from hemolysis, intercurrent infection, viral hepatitis (often transfusion associated), intrahepatic sickling (sickle cell hepatopathy), or choledocolithiasis (in 50-70% of adults; from pigment stones). [1] Sickle cell hepatopathy is an uncommon complication characterized by variable hyperbilirubinemia and either mild or severe hepatic dysfunction. [2] It ranges from the self-limiting hepatic right upper quadrant syndrome to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. [2],[3] Acute sickling episodes selectively affect the liver in 10% of patients, causing hepatic crisis with abdominal pain, nausea, fever, jaundice and transaminase elevation. [4] Exchange transfusion may be the only effective management for initial episodes. [4] Chronic intrahepatic cholestasis is an uncommon complication often punctuated by superimposed acute sickling episodes resembling acute viral hepatitis. [5] The histological consequences of intrahepatic sickling include impaction of hepatic sinusoids with sickled erythrocytes, patchy areas of hepatocellular necrosis, engorgement of Kupffer cells and bile stasis. [6] In children, manifestations of sickle cell hepatopathy are mild and include right upper quadrant pain, hepatomegaly, fever and leukocytosis, mild elevation of serum transaminases and moderate to marked elevation of serum bilirubin and alkaline phosphatase levels. [2] Although the course in children is benign and symptoms usually resolve in 1-3 weeks, progression to fulminant hepatic failure and death is more frequent in adults and occasionally seen in adolescence. [2] Prompt exchange transfusion and occasionally chronic transfusion have been the only effective therapies. [4],[5]

This patient of homozygous sickle cell disease had presented with hepatocellular jaundice due to repeated intrahepatic sickling episodes superimposed on more indolent cholestasis. Presentation with repeated attacks of abdominal pain and jaundice, conjugated hyperbilirubinemia, elevated liver enzymes together with histological features of intrahepatic cholestasis, portal inflammation and sinusoidal dilatation made the diagnosis clear. Because of this unique presentation, a possibility of hemolytic anemia was never considered unless an elevated reticulocyte count was noticed together with multiple gall stones. The diagnosis was established at the age of 26 years and he never had any of the commonly encountered features of homozygous sickle cell disease.

   References Top

1.Schubert TT. Hepatobiliary system in sickle cell disease. Gastroenterology 1986;90:2013-21.  Back to cited text no. 1  [PUBMED]  
2.Ahn H, Li CS, Wang W. Sickle cell hepatopathy. Pediatr Blood Cancer 2005;45:184-90.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Altintas E, Tiftik EN, Ucbilek E, Sezgin O. Sickle cell anemia connected with chronic intrahepatic cholestasis: A case report. Turk J Gastroenterol 2003;14:215-8.  Back to cited text no. 3    
4.Norris WE. Acute hepatic sequestration in sickle cell disease. J Natl Med Assoc 2004;96:1235-9.  Back to cited text no. 4  [PUBMED]  
5.Shao SH, Orringer EP. Sickle cell intrahepatic cholestasis: Approach to a difficult problem. Am J Gastroenterol 1995;90:2048-50.  Back to cited text no. 5  [PUBMED]  
6.Teixeira AL, Viana MB, Roquete ML, Toppa NH. Sickle cell disease: A clinical and histopathologic study of the liver in living children. J Pediatr Hematol Oncol 2002;24:125-9.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]

Correspondence Address:
Ranjana Bandyopadhyay
1B/3, Uttarpara Housing Estate, 88B, G T Road, Bhadrakali, Hooghly, West Bengal - 712 232
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.41698

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  [Figure 1], [Figure 2], [Figure 3]

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