Nakao Hiro, Ishiguro Akira, Ikoma Nahoko, Nishi Kentaro, Su Chemin, Nakadate Hisaya, Kubota Mitsuru, Hayakawa Masaki, Matsumoto Masanori
Department of General Pediatrics Department of Postgraduate Education and Training Division of Hematology, National Center for Child Health and Development, Okura, Setagaya-ku, Tokyo Department of Blood Transfusion Medicine, Nara Medical University, Shijo-cho, Kashihara, Nara, Japan.
Medicine (Baltimore). 2017 Apr;96(14):e6547. doi: 10.1097/MD.0000000000006547.
Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare.
A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia.
Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level.
INTERVENTIONS & OUTCOMES: Fresh frozen plasma was initially effective. Prednisolone and immunoglobulin resolved the condition with no adverse effects and rendered plasma exchange unnecessary.
Compared with biological agents, immunoglobulin is cost-effective, readily available, and has a proven long-term safety record, making it a possible treatment option for acquired thrombotic thrombocytopenic purpura.
血浆置换是获得性血栓性血小板减少性紫癜（TTP）的主要治疗方法，但具有侵入性且可能有不良反应。关于在不进行血浆置换的情况下使用免疫球蛋白治疗获得性TTP的报道很少。
一名14岁女孩因溶血性贫血和血小板减少入院。
根据低ADAMTS13（一种含血小板反应蛋白基序的解整合素样金属蛋白酶，13）活性和高ADAMTS13抑制物水平诊断为获得性TTP。
新鲜冷冻血浆最初有效。泼尼松龙和免疫球蛋白使病情得到缓解且无不良反应，无需进行血浆置换。
与生物制剂相比，免疫球蛋白具有成本效益、易于获得且有经证实的长期安全记录，使其成为获得性血栓性血小板减少性紫癜的一种可能治疗选择。
