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一例周期性无巨核细胞性血小板减少性紫癜所揭示的巨核细胞量与血清血小板生成素水平之间的关系

Relationship between megakaryocyte mass and serum thrombopoietin levels as revealed by a case of cyclic amegakaryocytic thrombocytopenic purpura.

作者信息

Zent C S, Ratajczak J, Ratajczak M Z, Anastasi J, Hoffman P C, Gewirtz A M

机构信息

Section of Hematology/Oncology, Department of Internal Medicine, University of Chicago, Illinois, USA.

出版信息

Br J Haematol. 1999 May;105(2):452-8.

Abstract

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.

摘要

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。

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