Hogland H C, Goldstein N P
Mayo Clin Proc. 1978 Aug;53(8):498-500.
The records of 54 consecutive patients with Wilson's disease seen at the Mayo Clinic from 1952 through early 1977 were reviewed to determine the frequency fo hematologic abnormalaties in their evaluation. Leukopenia and thrombocytopenia sometimes have been ascribed to treatment with D-penicillamine and its toxicity; however, we have found cytopenia to be a frequent finding in the presenting laboratory data of patients with Wilson's disease. Twenty-eight patients (52%) had thrombocytopenia and 16 of these patients (30% of the total) also had leukopenia. Severe, acute, intermittent hemolytic episodes were the initial and only presenting complaint of one patient. Six of the patients with significant cytopenias had splenectomy, and in all cases the peripheral blood counts returned to normal values. Long-term treatment with D-penicillamine improved the hepatic and neurologic dysfunction in most patients; however, the cytopenias remained unchanged except in three patients (treated 2, 5, and 10 years).
回顾了1952年至1977年初在梅奥诊所就诊的54例连续性威尔逊病患者的记录，以确定其评估中血液学异常的发生率。白细胞减少症和血小板减少症有时被归因于青霉胺治疗及其毒性；然而，我们发现血细胞减少症在威尔逊病患者的初始实验室数据中是常见的发现。28例患者（52%）有血小板减少症，其中16例患者（占总数的30%）也有白细胞减少症。严重、急性、间歇性溶血发作是1例患者的首发且唯一主诉。6例有明显血细胞减少症的患者接受了脾切除术，所有病例外周血细胞计数均恢复正常。大多数患者长期使用青霉胺治疗可改善肝脏和神经功能障碍；然而，除3例患者（治疗2年、5年和10年）外，血细胞减少症保持不变。
