2021
DOI: 10.9734/jpri/2021/v33i55b33850
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A Case of Wilson’s Disease Presenting with Persistant Hemolysis

Abstract: Wilson’s disease is one the rare autosomal recessive disorders of copper metabolism due to mutation in ATP7B gene located in chromosome 13. The mutations of this gene cause accumulation of copper in different tissues such as brain, liver, and eyes. The clinical presentation usually reflects this tissue distribution and varies from asymptomatic patients to those with hepatic or neuro-psychiatric manifestations. Here, we report an interesting case of Wilson’s disease which presented with mild persistent hemolysi… Show more

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“…The haematological manifestations of the disease include pancytopenia, isolated cell line dysfunction secondary to hypersplenism, and Coombs negative hemolytic anaemia [ 6 ]. The prevalence of hemolysis in patients of Wilson's disease is 17% which is due to the release of unbound copper from the dysfunctional hepatocytes leading to the oxidation of haemoglobin resulting in extravascular hemolysis of red blood cells (RBCs) [ 13 ]. However, no cases of Coombs positive hemolytic anaemia in Wilson’s disease have been reported.…”
Section: Discussionmentioning
confidence: 99%
“…The haematological manifestations of the disease include pancytopenia, isolated cell line dysfunction secondary to hypersplenism, and Coombs negative hemolytic anaemia [ 6 ]. The prevalence of hemolysis in patients of Wilson's disease is 17% which is due to the release of unbound copper from the dysfunctional hepatocytes leading to the oxidation of haemoglobin resulting in extravascular hemolysis of red blood cells (RBCs) [ 13 ]. However, no cases of Coombs positive hemolytic anaemia in Wilson’s disease have been reported.…”
Section: Discussionmentioning
confidence: 99%