Ueno Shikiko, Nozaki Junko, Kusano Shinichi, Kawano Yawara, Kikukawa Yoshitaka, Okuno Yutaka, Hata Hiroyuki, Matsuoka Masao, Uchiba Mitsuhiro
Department of Hematology, Rheumatology, and Infectious Disease, Kumamoto University Hospital.
LSI Medience Corporation.
Rinsho Ketsueki. 2019;60(10):1443-1448. doi: 10.11406/rinketsu.60.1443.
Coagulation factor XIII is a fibrin-stabilizing factor that leads to the crosslinking of fibrin when activated by thrombin. Acquired factor XIII inhibitor is caused when antibodies are generated against factor XIII, reducing its activity. Here we report a case of acquired factor XIII inhibitor. Although prednisolone was administered, factor XIII activity was not recovered. Interestingly, the activity normalized following the onset of multiple myeloma. The presence of inhibitors was evaluated in the patient's plasma by absorption tests and enzyme-linked immunosorbent assay. Immunoglobulin G inhibitors of factor XIII were present at admission, but later decreased with the onset of the IgA-λ-type myeloma. Thus, it is possible that the level of factor XIII inhibitors and polyclonal immunoglobulins could have been suppressed by the progression of myeloma, resulting in the normalization of factor XIII activity.
凝血因子 XIII 是一种纤维蛋白稳定因子，在被凝血酶激活时会导致纤维蛋白交联。获得性因子 XIII 抑制剂是在产生针对因子 XIII 的抗体时引起的，会降低其活性。在此我们报告一例获得性因子 XIII 抑制剂病例。尽管给予了泼尼松龙，但因子 XIII 活性并未恢复。有趣的是，在多发性骨髓瘤发病后活性恢复正常。通过吸收试验和酶联免疫吸附测定法对患者血浆中的抑制剂进行了评估。入院时存在因子 XIII 的免疫球蛋白 G 抑制剂，但后来随着 IgA-λ 型骨髓瘤的发病而降低。因此，有可能骨髓瘤的进展抑制了因子 XIII 抑制剂和多克隆免疫球蛋白的水平，从而导致因子 XIII 活性恢复正常。
