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.