First report of real-time monitoring of coagulation function potential and IgG subtype of anti-FVIII autoantibodies in a child with acquired hemophilia A associated with streptococcal infection and amoxicillin
First report of real-time monitoring of coagulation function potential and IgG subtype of anti-FVIII autoantibodies in a child with acquired hemophilia A associated with streptococcal infection and amoxicillin
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DOI：
10.1007/s12185-017-2273-6
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发表时间：
2017
影响因子：
2.1
通讯作者：
M. Takeyama;K. Nogami;Takahiro Kajimoto;K. Ogiwara;Tomoko Matsumoto;M. Shima
中科院分区：
文献类型：
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作者：
M. Takeyama;K. Nogami;Takahiro Kajimoto;K. Ogiwara;Tomoko Matsumoto;M. Shima
我们描述了一个8岁的男孩获得性血友病A（AHA）与链球菌感染和阿莫西林。实验室数据显示因子VIII活性（FVIII：C，1.5 IU/dl）和FVIII抑制剂（15.9 BU/ml）较低。综合凝血功能测定，包括旋转血栓弹性测定法（ROTEM®），显示凝血时间显著延长。凝血酶和纤溶酶生成（TG/PG）出现中度受损。他的抗FVIII自身抗体的抑制剂表位识别轻链和重链。患者接受Novoseven®和泼尼松龙治疗，病情迅速好转。ROTEM显示第20天凝血时间恢复至正常水平，TG逐渐改善。PG在临床早期中度降低，但在第20天改善。患者发病时IgG亚型为IgG 4。IgG 1在第20天呈一过性阳性，但在第46天呈阴性。FVIII抑制物逐渐减少，沿着FVIII：C升高，46天后完全消失。IgG 4在第83天再次升高，随后迅速降低，表明存在非中和抗体，目前仍未检测到。我们首次报道了一例罕见的AHA患儿的综合凝血功能和抗FVIII抗体IgG亚型的变化。
We describe an 8-year-old boy with acquired hemophilia A (AHA) associated with streptococcal infection and amoxicillin. Laboratory data revealed low factor VIII activity (FVIII:C, 1.5 IU/dl), and FVIII inhibitor (15.9 BU/ml). Comprehensive coagulation function assays, including rotation thromboelastometry (ROTEM®), revealed a markedly prolonged clotting time. Thrombin and plasmin generation (TG/PG) appeared to be moderately impaired. The inhibitor epitope of his anti-FVIII autoantibody recognized light and heavy chains. He was treated with Novoseven®and prednisolone, resulting in rapid improvement. ROTEM showed the return of coagulation time to normal level on day 20, and TG gradually improved. PG was moderately reduced in the clinical early phase, but improved at day 20. The patient’s IgG subtype was IgG4at onset. IgG1was transiently positive on day 20, but negative on day 46. FVIII inhibitor gradually decreased and was completely absent after day 46, along with the elevated FVIII:C. IgG4 was again elevated on day 83, followed by a rapid decrease, indicative of the presence of non-neutralizing antibody, which remains currently undetected. We for the first time report changes in comprehensive coagulation function and IgG subtype of anti-FVIII antibody in a rare pediatric case of AHA.