Skip to main content
Top
Published in: Archives of Gynecology and Obstetrics 1/2010

01-01-2010 | Case Report

Clinical features of hemolysis, elevated liver enzymes, and low platelet count syndrome in undiagnosed Wilson disease: report of two cases

Authors: Anna Członkowska, Grażyna Gromadzka, Janine Büttner, Grzegorz Chabik

Published in: Archives of Gynecology and Obstetrics | Issue 1/2010

Login to get access

Abstract

Introduction

Wilson’s disease (WD) is an autosomal recessive disorder characterized by toxic accumulation of copper mainly in the liver and brain. The hepatic manifestation of WD is diverse and may include asymptomatic elevation of aminotransferase, chronic hepatitis, cirrhosis, or acute/fulminant hepatic failure. Characteristic of acute hepatic failure in WD is concomitance of acute intravascular hemolytic anemia that in some patients may represent a first clinical symptom of WD. The diagnosis of acute Wilsonian liver failure is difficult, as similar signs may be observed in other clinical conditions. In pregnant patients with unrecognized WD, liver failure with hemolysis may be interpreted as the low platelet count (HELLP) syndrome.

Patients

We describe two women, who developed the clinical features of hemolysis, elevated liver enzymes, and HELLP syndrome. In both, further diagnostics confirmed WD.

Conclusion

WD should be remembered in the differential diagnostics of HELLP syndrome.
Literature
4.
go back to reference McCullough AJ, Fleming CR, Thistle JL et al (1983) Diagnosis of Wilson’s disease presenting as fulminant hepatic failure. Gastroenterology 84:161–167PubMed McCullough AJ, Fleming CR, Thistle JL et al (1983) Diagnosis of Wilson’s disease presenting as fulminant hepatic failure. Gastroenterology 84:161–167PubMed
6.
go back to reference Lee JJ, Kim HJ, Chung IJ et al (1998) Acute hemolytic crisis with fulminant hepatic failure as the first manifestation of Wilson’s disease: a case report. J Korean Med Sci 13:548–550PubMed Lee JJ, Kim HJ, Chung IJ et al (1998) Acute hemolytic crisis with fulminant hepatic failure as the first manifestation of Wilson’s disease: a case report. J Korean Med Sci 13:548–550PubMed
8.
go back to reference Balkema S, Hamaker ME, Visser HP et al (2008) Haemolytic anaemia as a first sign of Wilson’s disease. Neth J Med 66:344–347PubMed Balkema S, Hamaker ME, Visser HP et al (2008) Haemolytic anaemia as a first sign of Wilson’s disease. Neth J Med 66:344–347PubMed
9.
go back to reference Goldman M, Ali M (1991) Wilson’s disease presenting as Heinz-body hemolytic anemia. CMAJ 145:971–972PubMed Goldman M, Ali M (1991) Wilson’s disease presenting as Heinz-body hemolytic anemia. CMAJ 145:971–972PubMed
12.
go back to reference Sallie R, Katsiyiannakis L, Baldwin D et al (1992) Failure of simple biochemical indexes to reliably differentiate fulminant Wilson’s disease from other causes of fulminant liver failure. Hepatology 16:1206–1211CrossRefPubMed Sallie R, Katsiyiannakis L, Baldwin D et al (1992) Failure of simple biochemical indexes to reliably differentiate fulminant Wilson’s disease from other causes of fulminant liver failure. Hepatology 16:1206–1211CrossRefPubMed
13.
go back to reference Nazer H, Larcher VF, Ede RJ et al (1983) Wilson’s disease: a diagnostic dilemma. Br Med J (Clin Res Ed) 287:313–314CrossRef Nazer H, Larcher VF, Ede RJ et al (1983) Wilson’s disease: a diagnostic dilemma. Br Med J (Clin Res Ed) 287:313–314CrossRef
14.
go back to reference Korman JD, Volenberg I, Balko J et al (2008) Pediatric and Adult Acute Liver Failure Study Groups. Screening for Wilson disease in acute liver failure: a comparison of currently available diagnostic tests. Hepatology 48:1167–1174. doi:10.1002/hep.22446 CrossRefPubMed Korman JD, Volenberg I, Balko J et al (2008) Pediatric and Adult Acute Liver Failure Study Groups. Screening for Wilson disease in acute liver failure: a comparison of currently available diagnostic tests. Hepatology 48:1167–1174. doi:10.​1002/​hep.​22446 CrossRefPubMed
15.
go back to reference Walshe JM (1978) Don’t forget Wilson’s disease. BMJ 2:1384–1385 (Editorial) Walshe JM (1978) Don’t forget Wilson’s disease. BMJ 2:1384–1385 (Editorial)
16.
go back to reference Mihu D, Costin N, Mihu CM et al (2007) Seicean A, Ciortea R. HELLP syndrome—a multisystemic disorder. J Gastrointestin Liver Dis 16:419–424 Mihu D, Costin N, Mihu CM et al (2007) Seicean A, Ciortea R. HELLP syndrome—a multisystemic disorder. J Gastrointestin Liver Dis 16:419–424
17.
go back to reference Sibai BM, Taslimi MM, el-Nazer A et al (1986) Maternal-perinatal outcome associated with the syndrome of hemolysis, elevated liver enzymes, and low platelets in severe preeclampsia-eclampsia. Am J Obstet Gynecol 155:501–509PubMed Sibai BM, Taslimi MM, el-Nazer A et al (1986) Maternal-perinatal outcome associated with the syndrome of hemolysis, elevated liver enzymes, and low platelets in severe preeclampsia-eclampsia. Am J Obstet Gynecol 155:501–509PubMed
20.
go back to reference Sibai BM (2004) Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol 103:981–991PubMed Sibai BM (2004) Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol 103:981–991PubMed
22.
go back to reference Wakim-Fleming J, Zein NN (2005) The liver in pregnancy: disease vs benign changes. Cleve Clin J Med 72:713–721CrossRefPubMed Wakim-Fleming J, Zein NN (2005) The liver in pregnancy: disease vs benign changes. Cleve Clin J Med 72:713–721CrossRefPubMed
23.
go back to reference Mihu D, Costin N, Mihu CM et al (2007) HELLP syndrome—a multisystemic disorder. J Gastrointestin Liver Dis 16:419–424PubMed Mihu D, Costin N, Mihu CM et al (2007) HELLP syndrome—a multisystemic disorder. J Gastrointestin Liver Dis 16:419–424PubMed
26.
go back to reference Kato GJ, McGowan V, Machado RF et al (2006) Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood 107:2279–2285. doi:10.1182/blood-2005-06-2373 CrossRefPubMed Kato GJ, McGowan V, Machado RF et al (2006) Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood 107:2279–2285. doi:10.​1182/​blood-2005-06-2373 CrossRefPubMed
27.
go back to reference Berman DH, Leventhal RI, Gavaler JS et al (1991) Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure. Gastroenterology 100:1129–1134PubMed Berman DH, Leventhal RI, Gavaler JS et al (1991) Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure. Gastroenterology 100:1129–1134PubMed
32.
go back to reference Hogland HC, Goldstein NP (1978) Hematologic (cytopenic) manifestations of Wilson’s disease. Mayo Clin Proc 53:498–500PubMed Hogland HC, Goldstein NP (1978) Hematologic (cytopenic) manifestations of Wilson’s disease. Mayo Clin Proc 53:498–500PubMed
34.
38.
go back to reference Schagen van Leeuwen JH, Christiaens GC, Hoogenraad TU (1991) Recurrent abortion and the diagnosis of Wilson disease. Obstet Gynecol 78:547–549PubMed Schagen van Leeuwen JH, Christiaens GC, Hoogenraad TU (1991) Recurrent abortion and the diagnosis of Wilson disease. Obstet Gynecol 78:547–549PubMed
39.
go back to reference Shimono N, Ishibashi H, Ikematsu H et al (1991) Fulminant hepatic failure during perinatal period in a pregnant woman with Wilson’s disease. Gastroenterol Jpn 26:69–73PubMed Shimono N, Ishibashi H, Ikematsu H et al (1991) Fulminant hepatic failure during perinatal period in a pregnant woman with Wilson’s disease. Gastroenterol Jpn 26:69–73PubMed
Metadata
Title
Clinical features of hemolysis, elevated liver enzymes, and low platelet count syndrome in undiagnosed Wilson disease: report of two cases
Authors
Anna Członkowska
Grażyna Gromadzka
Janine Büttner
Grzegorz Chabik
Publication date
01-01-2010
Publisher
Springer-Verlag
Published in
Archives of Gynecology and Obstetrics / Issue 1/2010
Print ISSN: 0932-0067
Electronic ISSN: 1432-0711
DOI
https://doi.org/10.1007/s00404-009-1080-6

Other articles of this Issue 1/2010

Archives of Gynecology and Obstetrics 1/2010 Go to the issue