Hirsh E H, Vogler W R, McDonald T P, Stein S F
Arch Intern Med. 1980 May;140(5):721-3. doi: 10.1001/archinte.140.5.721.
A 49-year-old woman had purpura and thrombocytopenia not associated with drugs or identifiable underlying disease. The platelet survival was normal and the marrow showed a sharp reduction in megakaryocytes with preservation of other cell lines. There was no response to steroids or infusion of fresh frozen plasma. Lithium carbonate therapy similarly had no effect. Thrombopoietic activity was absent in serum and urine samples. Erythropoietin activity was normal. In vitro formation of granulocyte-macrophage colonies in soft agar was normal. The case represents a unique incidence of selective megakaryocytic hypoplasia, though to result from a failure in stem cell differentiation.
一名49岁女性出现紫癜和血小板减少症,与药物或可识别的潜在疾病无关。血小板生存期正常,骨髓显示巨核细胞急剧减少,其他细胞系则得以保留。使用类固醇或输注新鲜冷冻血浆均无反应。碳酸锂治疗同样无效。血清和尿液样本中不存在血小板生成活性。促红细胞生成素活性正常。软琼脂中粒细胞-巨噬细胞集落的体外形成正常。该病例代表了一种独特的选择性巨核细胞发育不全的情况,尽管是由干细胞分化失败导致的。