Ganti Apar Kishor, Vose Julie M, Haire William D
Department of Internal Medicine, Section of Oncology-Hematology, University of Nebraska Medical Center, 987680 Nebraska Medical Center, Omaha, NE 68198-7680, USA.
J Thromb Thrombolysis. 2007 Apr;23(2):155-8. doi: 10.1007/s11239-006-9037-0.
Dysfibrinogenemia is a disorder of fibrinogen structure and is associated with a functional abnormality. Since fibrinogen is a key component of both the procoagulant and fibrinolytic pathways, defects in fibrinogen function can be associated with increased risk for both hemorrhage and thrombosis. Management of patients with dysfibrinogenemia and a thrombotic tendency usually involves long-term anticoagulation.
A 36-year-old male with relapsed nodular sclerosing Hodgkin's was found to have a prolonged prothrombin time, low fibrinogen activity and a normal fibrinogen antigen during evaluation for a hematopoietic peripheral blood stem cell transplant. His past medical history was significant for an acute myocardial infarction and two episodes of acute pancreatitis. His father had dysfibrinogenemia complicated by multiple thrombotic episodes. A trans-esophageal echocardiogram revealed two thrombi, one each in the superior vena cava and the descending aorta. He was treated with enoxaparin and received peripheral blood stem cell transplantation. An effort was made to maintain his fibrinogen activity levels at 200 mg/dL using cryoprecipitate. A month following the transplant he developed a new thrombus in the right internal jugular vein, while on enoxaparin and he was started on argatroban and cryoprecipitate followed by fondaparinux. A repeat echocardiogram six weeks later demonstrated that the burden of thrombus both in the right atrium and descending aorta was significantly lower.
This is the first case report of a patient with dysfibrinogenemia undergoing peripheral blood stem cell transplantation. Conventional anticoagulant therapy and cryoprecipitate seem to be a reasonable management strategy to prevent thrombosis in a patient with dysfibrinogenemia and a thrombophilic tendency. Secondly, fondaparinux can be used in cases of failure of therapy with low molecular weight heparins and may actually be superior to low molecular weight heparins, especially in patients with dysfibrinogenemia.
异常纤维蛋白原血症是一种纤维蛋白原结构紊乱的疾病，与功能异常相关。由于纤维蛋白原是凝血和纤溶途径的关键成分，纤维蛋白原功能缺陷可导致出血和血栓形成风险增加。异常纤维蛋白原血症且有血栓形成倾向的患者的管理通常涉及长期抗凝。
一名36岁复发性结节硬化型霍奇金淋巴瘤男性患者，在接受造血外周血干细胞移植评估时，发现其凝血酶原时间延长、纤维蛋白原活性降低而纤维蛋白原抗原正常。他既往有急性心肌梗死病史和两次急性胰腺炎发作史。他的父亲患有异常纤维蛋白原血症并伴有多次血栓形成发作。经食管超声心动图显示有两个血栓，分别位于上腔静脉和降主动脉。他接受了依诺肝素治疗并接受了外周血干细胞移植。使用冷沉淀努力将他的纤维蛋白原活性水平维持在200mg/dL。移植后一个月，他在接受依诺肝素治疗时右颈内静脉出现新的血栓，随后开始使用阿加曲班和冷沉淀，之后使用磺达肝癸钠。六周后重复超声心动图显示右心房和降主动脉的血栓负荷均显著降低。
这是首例关于异常纤维蛋白原血症患者接受外周血干细胞移植的病例报告。传统抗凝治疗和冷沉淀似乎是预防异常纤维蛋白原血症且有血栓形成倾向患者血栓形成的合理管理策略。其次，在低分子量肝素治疗失败的情况下可使用磺达肝癸钠，其可能实际上优于低分子量肝素，尤其是在异常纤维蛋白原血症患者中。
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