Sato Atsushi, Hoshi Yoshiyuki, Onuma Masaei, Sato Ryusuke, Tsunematsu Yukiko, Isonishi Ayami, Matsumoto Masanori, Fujimura Yoshihiro, Imaizumi Masue
Department of Hematology and Oncology, Miyagi Children's Hospital, Aoba-ku, Sendai, Japan.
Pediatr Hematol Oncol. 2010 Feb;27(1):53-8. doi: 10.3109/08880010903401752.
Although acquired idiopathic thrombotic thrombocytopenic purpura (ai-TTP) is rare in children, the authors present the case of a 9-month-old boy with ai-TTP showing severe deficiency of ADAMTS13 activity by its inhibitory IgG-autoantibody (4.8 Bethesda units/mL). Plasma exchange therapy was clinically effective but transient. Deficient activity of ADAMTS13 with the presence of its inhibitor persisted for 7 months after the initial diagnosis. However, other laboratory findings improved gradually with steroid (pulse) therapy. The hitherto insufficiently characterized clinical settings of ai-TTP during early childhood underscore the importance of measuring ADAMTS13 activity and its inhibitors for differential diagnosis in patients with thrombocytopenia of unknown etiology.
尽管获得性特发性血栓性血小板减少性紫癜（ai-TTP）在儿童中较为罕见，但作者报告了一例9个月大患有ai-TTP的男童，其ADAMTS13活性因抑制性IgG自身抗体而严重缺乏（4.8贝塞斯达单位/毫升）。血浆置换疗法临床有效但为一过性。初始诊断后，ADAMTS13活性缺乏及其抑制剂持续存在了7个月。然而，其他实验室检查结果经类固醇（冲击）治疗后逐渐改善。幼儿期ai-TTP迄今特征描述不足的临床情况凸显了在病因不明的血小板减少症患者中检测ADAMTS13活性及其抑制剂以进行鉴别诊断的重要性。
