Abstract
Splenomegaly and pancytopenia are common in Wilson’s disease (WD) and splenectomy is one of the conventional treatments for splenomegaly and the associated pancytopenia. However, splenectomy remained controversial for hypersplenism in WD as it was reported that splenectomy leaded to serious emotional and neurological deterioration in WD patients with hypersplenism. In the current study, we present our experiences in 70 WD patients with hypersplenism who had undergone splenectomy, outlining the safety and efficacy of splenectomy in WD. The clinical database of 70 WD patients with hypersplenism who had undergone splenectomy in our hospital between 2009 and 2013 were reviewed and followed-up regularly. Before splenectomy, all the patients accepted a short period of anti-copper treatment with intravenous sodium 2, 3-dimercapto-1-propane sulfonate (DMPS). All the patients demonstrated a marked improvement in platelet and leucocyte counts after splenectomy. No severe postoperative complication was observed. In particular, none of the 37 patients with mixed neurologic and hepatic presentations experienced neurological deterioration after splenectomy, and none of the patients with only hepatic presentations newly developed neurological symptoms. During the one year follow-up period, no patient presented hepatic failure or hepatic encephalopathy, no hepatic patient newly developed neurological presentations, and only 3 patients with mixed neurologic and hepatic presentations suffered neurological deterioration and these 3 patients had poor compliance of anti-copper treatment. Quantative analysis of the neurological symptoms in the 37 patients using the Unified Wilson’s Disease Rating Scale (UWDRS) showed that the neurological symptoms were not changed in a short-term of one week after splenectomy but significantly improved in a long-term of one year after splenectomy. Additionally, compared to that before splenectomy, the esophageal gastric varices in most patients significantly improved one year after splenectomy. Thus, we may conclude that splenectomy is a safe and effective therapeutic measure for hypersplenism in WD patients who had been preoperatively treated with DMPS for powerful anti-copper therapy.
Highlights
Wilson’s disease (WD) is an autosomal recessive disorder of copper metabolism which results in pathological accumulation of copper in various tissues and organs, predominantly in the liver, brain, and cornea
We present our experiences of splenectomy for hypersplenism in 70 WD patients, outlining its safety and efficacy
The white blood cell (WBC) and PLT counts in WD patients with hypersplenism were obviously lower than their normal ranges (WBC count, 4–10×109; PLT, 100–300×109) (Table 3)
Summary
Wilson’s disease (WD) is an autosomal recessive disorder of copper metabolism which results in pathological accumulation of copper in various tissues and organs, predominantly in the liver, brain, and cornea. Toxic copper accumulation in these tissues and organs contribute to the neurological, psychiatric and hepatic symptoms including that of acute or chronic hepatitis, fulminant hepatic failure, and cirrhosis [1,2,3,4]. Hypersplenism is clinically characterized by splenomegaly, thrombocytopenia, leukopenia, and anemia[5]. Thrombocytopenia and leucopenia increase the risk of spontaneous bleeding and bacterial infections. Some anti-copper drugs, such as penicillamine, trientine and tetrathiomolybdate can occasionally cause bone marrow depression, anemia, leucopenia, immunological lesions, and even paradoxical symptom worsening[6,7,8]. Anti-copper therapy must often be stopped in patients with WD, which invariably leads to progression of the disease [2,4]
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