Kotlín Roman, Sobotková Alzbeta, Riedel Tomás, Salaj Peter, Suttnar Jirí, Reicheltová Zuzana, Májek Pavel, Khaznadar Tarek, Dyr Jan E
Institute of Hematology and Blood Transfusion, Praha, Czech Republic.
Acta Haematol. 2008;120(2):75-81. doi: 10.1159/000160182. Epub 2008 Oct 8.
Abnormal coagulation properties indicative of a dysfibrinogen were found in the plasma of a 72-year-old male with multiple myeloma (IgGkappa, stage IIIA). The patient had high paraprotein concentration (85.75 g/l) and prolonged thrombin time (76.8 s), activated partial thromboplastin time (39.5 s), prothrombin time (23.5 s) and reptilase time (72.0 s). The fibrinogen level was increased. The fibrin polymerization induced by both thrombin and reptilase was impaired. Scanning electron microscopy revealed abnormal clot morphology. After six months of treatment, the paraprotein level decreased (19.48 g/l) and coagulation normalized as well as fibrin polymerization and fibrin clot morphology. It was found that the paraprotein interacts with the gamma-chain of fibrinogen. Acquired dysfibrinogenemia associated with multiple myeloma was diagnosed in the 72-year-old patient.
在一名患有多发性骨髓瘤（IgGκ，ⅢA期）的72岁男性患者的血浆中发现了提示异常纤维蛋白原血症的异常凝血特性。该患者副蛋白浓度高（85.75 g/l），凝血酶时间延长（76.8秒），活化部分凝血活酶时间（39.5秒），凝血酶原时间（23.5秒）和蛇毒凝血酶时间（72.0秒）。纤维蛋白原水平升高。凝血酶和蛇毒凝血酶诱导的纤维蛋白聚合均受损。扫描电子显微镜显示凝块形态异常。经过六个月的治疗，副蛋白水平下降（19.48 g/l），凝血功能恢复正常，纤维蛋白聚合和纤维蛋白凝块形态也恢复正常。发现副蛋白与纤维蛋白原的γ链相互作用。该72岁患者被诊断为与多发性骨髓瘤相关的获得性异常纤维蛋白原血症。
