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我院1960～1986年共收治肝豆状核变性80例,其中39例并有贫血,占48.8％。中重度贫血25例,占贫血病人的64.1％。39例中失血性贫血16例(包括胃肠道失血为主者10例,鼻衄和皮肤粘膜出血为主者5例,血尿1例),溶血性贫血7例,脾功能亢进性(脾亢)贫血5例,营养性贫血1例,原因未明者10例。溶血性贫血均发生在4个月内,其中3例贫血为肝豆状核变性的首发症状。失血性贫血及脾亢贫血均发病较晚。所有病人均给对症治疗,部分病人采用祛铜治疗。预后决定于原发病程度。溶血性贫血7例中3例并发肝功能衰竭。
Our hospital from 1960 to 1986 were treated 80 cases of Wilson’s disease, including 39 cases and anemia, accounting for 48.8%. 25 cases of moderate to severe anemia, accounting for 64.1% of patients with anemia. 39 cases of hemorrhagic anemia in 16 cases (including gastrointestinal bleeding in 10 cases, mainly in the epistaxis and mucocutaneous bleeding in 5 cases, hematuria in 1 case), hemolytic anemia in 7 cases, hypersplenism (hypersplenism ) Anemia in 5 cases, 1 case of nutritional anemia, unexplained 10 cases. Hemolytic anemia occurred in 4 months, of which 3 cases of anemia is the first symptom of hepatolenticular degeneration. Hemolytic anemia and anemia are relatively late. All patients were given symptomatic treatment, some patients with bronze therapy. The prognosis depends on the original degree of disease. Hemolytic anemia in 7 cases in 3 cases complicated by liver failure.