Kawano Hiroki, Suzuki Tomohide, Ishii Shinichi, Wakahashi Kanako, Kawano Yuko, Sada Akiko, Minagawa Kentaro, Takaya Tomofumi, Yamashita Tomoya, Hirata Ken-Ichi, Koriyama Kenji, Nagamatsu Yuichi, Matsui Toshimitsu, Katayama Yoshio
Department of Hematology, Kobe University Graduate School of Medicine.
Rinsho Ketsueki. 2014 Jun;55(6):697-702.
We report two patients (70- and 49-year-old Japanese men) with acute exacerbation of chronic idiopathic thrombocytopenic purpura (ITP) and deep venous thrombosis of the lower extremities. Both were successfully managed with thrombopoietin receptor agonist (TPO-RA) administration. Both had ITP refractory to steroid treatment. Their immature platelet fraction (absolute-IPF) counts were increased and paralleled the platelet recoveries after TPO-RA (eltrombopag and romiplostim, respectively) without progression of thrombosis. Although ITP has recently been evaluated as a thrombophilic disorder, reports on acute exacerbation of ITP with newly diagnosed thrombosis are limited, and the pathophysiology and association between ITP and thrombosis remain to be elucidated. Moreover, the influences of TPO-RA on thrombosis are still controversial. To our knowledge, this is the first case report describing patients with exacerbation of ITP who developed thrombosis and were treated with TPO-RA. The outcomes of our cases underscore the importance of monitoring thrombosis and not delaying the initiation of anticoagulation treatment during the use of TPO-RA.
我们报告了两名患有慢性特发性血小板减少性紫癜(ITP)急性加重并伴有下肢深静脉血栓形成的患者(分别为70岁和49岁的日本男性)。两者均通过给予血小板生成素受体激动剂(TPO-RA)成功治疗。两人的ITP对类固醇治疗均无效。他们的未成熟血小板分数(绝对未成熟血小板分数)计数增加,并且与TPO-RA(分别为艾曲泊帕和罗米司亭)治疗后的血小板恢复情况平行,且血栓形成未进展。尽管最近ITP被评估为一种易栓症,但关于新诊断血栓形成的ITP急性加重的报道有限,ITP与血栓形成之间的病理生理学及关联仍有待阐明。此外,TPO-RA对血栓形成的影响仍存在争议。据我们所知,这是首例描述ITP加重并发血栓形成并接受TPO-RA治疗患者的病例报告。我们病例的结果强调了在使用TPO-RA期间监测血栓形成以及不延迟启动抗凝治疗的重要性。