Elsevier

Clinics in Liver Disease

Volume 6, Issue 4, November 2002, Pages 891-907
Clinics in Liver Disease

Anemia and the liver: Hepatobiliary manifestations of anemia

https://doi.org/10.1016/S1089-3261(02)00050-8Get rights and content

Section snippets

Sickle cell anemia

Sickle cell anemia is an autosomal recessive condition that is present in 0.15% of black children in the United States and is caused by a mutation in the gene coding for the β-chain of hemoglobin. The mutation results in substitution of the normal gutamic acid by valine at amino acid 6 in the β-globin gene (often referred to as Glu6Val or E6V). Hemoglobin SS (HbSS) is unstable. It undergoes noncovalent polymerization in capillary or venous deoxygenated blood. Polymerized HbSS forms long strands

Paroxysmol nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria is associated with nearly continuous hemolysis, anemia, and intermittent hemoglobinuria, which usually increases during the night. Neutropenia and thrombocytopenia are also common. PNH usually is identified between ages 30 and 50 years [33], [34]. In some patients, the diagnosis is established before age 20 [33], [35] or in advanced age [33], [34]. Cell-membrane defects are present on erythrocytes, polymorphonuclear leukocytes, platelets, monocytes, and

G6PD deficiency

Glucose-6-phosphate dehydrogenase is an enzyme that catalyzes the first reaction in the pentose-phosphate pathway. It is the only source of reduced NADPH inside erythrocytes. NADPH in red blood cells reduces oxidized glutathione. The reduced form of glutathione is an important antioxidant, thiol doner, and cytoprotectant. Erythrocytes that have G6PD deficiency accumulate oxidized glutathione, which in turn leads to denaturation of hemoglobin and hemolysis after exposure to oxidative stress;

Hereditary spherocytosis

The most common heritable anemia in people of Northern European ancestry is hereditary spherocytosis, which affects about 1 of 5000 individuals in the United States and in the United Kingdom [56], [57]. Hereditary spherocytosis also occurs in nearly all racial groups. There are at least five genetic loci for gene mutations that can result in hereditary spherocytosis: α-spectrin on the long arm of chromosome 1 (1q21); ankyrin-1 on the short arm of chromosome 8 (8p11.2); β-spectrin on the long

HELLP syndrome

The HELLP syndrome is characterized by hemolysis, elevated liver enzymes, and low platelets. It occurs in the setting of late pregnancy and is associated with preeclampsia and eclampsia. Women whose blood pressure reaches or exceeds 140/90 mm Hg during pregnancy and decreases to normal within 6 weeks of delivery have gestational hypertension [83]. About 6% to 18% of pregnancies are associated with gestational hypertension [84], [85], [86], [87]. Asymptomatic individuals with gestational

First page preview

First page preview
Click to open first page preview
View PDF

References (110)

  • J. Takeda et al.

    Deficiency of the GPI anchor caused by somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria

    Cell

    (1993)
  • D. Valla et al.

    Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria: a spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome

    Gastroenterol

    (1987)
  • T. Vulliamy et al.

    Hematologically important mutations: glucose-6-phosphate dehydrogenase deficiency

    Blood Cells Mol Dis

    (1997)
  • E. Beutler

    G6PD: population genetics and clinical manifestations

    Blood Rev

    (1996)
  • S.W. Eber et al.

    Variable clinical severity of hereditary spherocytosis: relation to erythrocytic spectrim concentration, osmotic fragility, and autohemolysis

    J Pediatr

    (1990)
  • P.G. Gallagher et al.

    Hematologically important mutations: spectrin and ankyrin variants in hereditary spherocytosis

    Blood Cells Mol Dis

    (1998)
  • E. Miraglia del Giudice et al.

    High frequency of de novo mutations in ankyrin gene (ANK1) in children with hereditary spherocytosis

    J Pediatr

    (1998)
  • E.A. Evans et al.

    Elastic area compressability modulus of red cell membrane

    Biophys J

    (1976)
  • G.C. Bates et al.

    Incidence of gallbladder disease in chronic hemolytic anemia (spherocytosis)

    Gastroenterol

    (1952)
  • L.E. Young et al.

    Hereditary spherocytosis. I. Clinical, hematologic and genetic features in 28 cases, with particular reference to the osmotic and mechanical fragility of incubated erythrocytes

    Blood

    (1951)
  • D.N. Mohler et al.

    Hemochromatosis heterozygotes may have significant iron overload when they also have hereditary spherocytosis

    Am J Med Sci

    (1986)
  • M. Barry et al.

    Hereditary spherocytosis with secondary haemochromatosis

    Lancet

    (1968)
  • J.C. Hauth et al.

    Pregnancy outcomes in healthy nulliparas who developed hypertension: Calcium for Preeclampsia Prevention Study Group

    Obstet Gynecol

    (2000)
  • M. Knuist et al.

    Intensification of fetal and maternal surveillance in pregnant women with hypertensive disorders

    Int J Gynaecol Obstet

    (1998)
  • J.N. Martin et al.

    The natural history of HELLP syndrome: patterns of disease progression and regression

    Am J Obstet Gynecol

    (1991)
  • B.M. Sibai

    The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing?

    Am J Obstet Gynecol

    (1990)
  • L. Weinstein

    Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy

    Am J Obstet Gynecol

    (1982)
  • B.M. Sibai et al.

    Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome)

    Am J Obstet Gynecol

    (1993)
  • G. Wilke et al.

    Haptoglobin as a sensitive marker of hemolysis in HELLP syndrome

    Int J Gynaecol Obstet

    (1992)
  • P.L. Zusterzeel et al.

    Gilbert's syndrome is not associated with HELLP syndrome

    Br J Obstet Gynaecol

    (2001)
  • J.R. Barton et al.

    Hepatic imaging in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count)

    Am J Obstet Gynecol

    (1996)
  • J.R. Barton et al.

    Hepatic histopathologic condition does not correlate with laboratory abnormalities in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count)

    Am J Obstet Gynecol

    (1992)
  • F. Audibert et al.

    Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome

    Am J Obstet Gynecol

    (1996)
  • T.W. Green et al.

    The liver in sickle cell anemia

    Johns Hopkins Med J

    (1953)
  • H.J. Rosenblate et al.

    The liver in sickle cell anemia: a clinical-pathological study

    Arch Pathol

    (1970)
  • M. Brozovic et al.

    Acute admissions of patients with sickle cell disease who live in Britain

    BMJ

    (1987)
  • C.S. Johnson et al.

    Liver involvement in sickle cell disease

    Medicine (Baltimore)

    (1985)
  • D.R. Powars

    Natural history of sickle cell disease—the first ten years

    Semin Hematol

    (1975)
  • P. Antal et al.

    Is the incidence of apendicitis reduced in patients with sickle cell disease?

    Pediatrics

    (1998)
  • J.B. Herrick

    Peculiar elongated and sickle-shaped red corpuscles in a case of severe anemia

    Arch Intern Med

    (1910)
  • J. Karayalcin et al.

    Sickle cell anemia-clinical manifestations in 100 patients and review of the literature

    Am J Med Sci

    (1975)
  • M. Omata et al.

    Pathological spectrum of liver diseases in sickle cell disease

    Dig Dis Sci

    (1986)
  • S. Laulan et al.

    Systematic blood transfusions in adult homozygote sickle-cell anemia

    Presse Med

    (1990)
  • M.E. Conrad

    Sickle cell disease and hemochromatosis

    Am J Hematol

    (1991)
  • Y.S. Song

    Hepatic lesions in sickle cell anemia

    Am J Pathol

    (1957)
  • E. Barrett-Connor

    Sickle cell disease and viral hepatitis

    Ann Intern Med

    (1968)
  • J.C. Phillips et al.

    The incidence of cholelithiasis in sickle cell disease

    Am J Roentgenol

    (1971)
  • I.W. McCall et al.

    Cholelithiasis in Jamaican patients with homozygous sickle cell disease

    Am J Hematol

    (1977)
  • B.W. Trotman et al.

    Pigment gallstone disease: summary of the National Institutes of Health-international work-shop

    Hepatology

    (1982)
  • B.W. Trotman

    Insights into pigment stone disease

    J Lab Clin Med

    (1979)

    • Cited by (33)

    • Systemic disease

      2012, MacSween's Pathology of the Liver

    • Emergency Management of Red Blood Cell Disorders

      2012, Emergency Medicine: Clinical Essentials, SECOND EDITION

    • Systemic Disease

      2011, MacSween's Pathology of the Liver: Expert Consult: Online and Print

    • The blood in systemic disease

      2009, Medicine
      Citation Excerpt :

      Excessive alcohol causes macrocytosis in the absence of anaemia or overt liver damage. Sideroblastic anaemia and haemolytic anaemia may occur,7 including severe haemolysis in Zieve’s syndrome and Wilson’s disease. There is increased risk of bleeding from reduced synthesis of clotting factors and mild DIC can also occur.8

    • Glucose-6-phosphate dehydrogenase deficiency

      2008, The Lancet
      Citation Excerpt :

      The precise mechanism by which increased sensitivity to oxidative damage leads to haemolysis is not fully known; furthermore, the exact sequence of events once an exogenous trigger factor is present is also unknown. Whatever the cause of the acute haemolysis in G6PD deficiency, it is characterised clinically by fatigue, back pain, anaemia, and jaundice.90 Increased unconjugated bilirubin, lactate dehydrogenase, and reticulocytosis are markers of the disorder.

    • The Hematopoietic Effect of Red and White Peels Pomegranate Extract on Phenylhydrazine-Induced Anemia in Rats and LCMS/ MS investigations of new substances

      2022, Research Square
    View all citing articles on Scopus
    View full text
    Copyright © 2002 Elsevier Science (USA). All rights reserved.