Zent C S, Ratajczak J, Ratajczak M Z, Anastasi J, Hoffman P C, Gewirtz A M
Section of Hematology/Oncology, Department of Internal Medicine, University of Chicago, Illinois, USA.
Br J Haematol. 1999 May;105(2):452-8.
Cyclic amegakaryocytic thrombocytopenic purpura is a rare syndrome characterized by periodic failure of megakaryocytopoiesis. In this report we describe a patient with cyclic amegakaryocytic thrombocytopenic purpura associated with a megakaryocyte specific serum IgG antibody, who responded to cyclosporin A therapy. Serial serum thrombopoietin assays during an episode of platelet cycling demonstrated a reciprocal relationship between serum thrombopoietin level and megakaryocyte mass, suggesting that megakaryocytes have an important role in the regulation of thrombopoietin metabolism.
周期性无巨核细胞性血小板减少性紫癜是一种罕见的综合征,其特征为巨核细胞生成周期性衰竭。在本报告中,我们描述了一名患有与巨核细胞特异性血清IgG抗体相关的周期性无巨核细胞性血小板减少性紫癜的患者,该患者对环孢素A治疗有反应。在血小板循环发作期间进行的系列血清血小板生成素检测显示,血清血小板生成素水平与巨核细胞数量之间呈反比关系,提示巨核细胞在血小板生成素代谢调节中起重要作用。