Successful Splenectomy for Hypersplenism in Wilson’s Disease: A Single Center Experience from China

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.

Differential hepatic features presenting in Wilson disease-associated cirrhosis and hepatitis B-associated cirrhosis

“…Thrombocytopenia was defined as a platelet count < 100 × 10 9 /L. The indication for splenectomy was hypersplenism, characterized by splenomegaly, leukopenia, erythropenia, and thrombocytopenia[19]. Smokers were defined as patients who have smoked at some stages in their lifetime.…”

Combined dimercaptosuccinic acid and zinc treatment in neurological Wilson’s disease patients with penicillamine-induced allergy or early neurological deterioration

“…The diagnosis of WD was based on our previous standard [ 20 ], including characteristic clinical manifestations, positive family history, low serum ceruloplasmin (<0.2 g/l), elevated 24-h urinary copper (24-h UC) excretion (>100 µg/24 h), elevated liver copper (>250 µg/g dry weight), presence of a Kayser–Fleischer (K–F) ring, elevated 24-h UC excretion following the administration of 2× 500-mg doses of DPA (>1600 µg/24 h) and magnetic resonance imaging (MRI) of brain [ 21 , 22 ], and met the WD diagnostic criteria formed at the 8th International Meeting on Wilson’s disease [ 23 , 24 ].…”

Combined sodium Dimercaptopropanesulfonate and zinc versus D-penicillamine as first-line therapy for neurological Wilson’s disease

“…This enables the DMPS to excrete the extra copper through urine after filtration in the kidneys [9]. Although it is not widely popularized in other areas, DMPS has been universally used as a routine treatment for WD in China for several decades [10,11]. In addition, it is also commonly used as an alternative treatment for WD patients, who are intolerant to the DPA treatment [9,12].…”