Kim Peter Geon, Suh Joome, Adelman Max W, Oduro Kwadwo, Williams Erik, Brunner Andrew M, Kuter David J
Department of Medicine, Massachusetts General Hospital, Boston, MA 02114, USA.
Department of Pathology, Massachusetts General Hospital, Boston, MA 02114, USA.
Case Rep Hematol. 2016;2016:4161679. doi: 10.1155/2016/4161679. Epub 2016 Mar 17.
Pure white cell aplasia (PWCA) is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. PWCA has been associated with autoimmune, drug-induced, and viral exposures. Here, we report a case of a 74-year-old female who presented with severe proximal weakness without pain and was found to have PWCA with nonspecific inflammatory necrotizing myositis and acute liver injury on biopsies. These findings were associated with a recent course of azithromycin and her daily use of a statin. Myositis improved on prednisone but PWCA persisted. With intravenous immunoglobulin and granulocyte-colony stimulating factor therapies, her symptoms and neutrophil counts improved and were sustained for months.
纯白细胞再生障碍性贫血（PWCA）是一种罕见的血液系统疾病，其特征是骨髓中缺乏中性粒细胞系，而巨核细胞生成和红细胞生成正常。PWCA与自身免疫、药物诱导和病毒暴露有关。在此，我们报告一例74岁女性病例，该患者表现为严重的近端肌无力且无疼痛，活检发现患有PWCA，并伴有非特异性炎症坏死性肌炎和急性肝损伤。这些发现与近期使用阿奇霉素及她日常服用他汀类药物有关。肌炎在使用泼尼松后有所改善，但PWCA持续存在。通过静脉注射免疫球蛋白和粒细胞集落刺激因子治疗，她的症状和中性粒细胞计数得到改善，并持续了数月。
