Section of Hematology and Oncology, Boston University School of Medicine, Boston, MA, USA.
Amyloid. 2012 Sep;19(3):156-60. doi: 10.3109/13506129.2012.694825. Epub 2012 Jun 11.
A 41-year-old woman with immunoglobulin light chain (AL) systemic amyloidosis with cardiac and gastrointestinal involvement developed multiple arterial and venous thromboembolic complications. Treatment with unfractionated heparin was complicated by life-threatening gastrointestinal bleeding. Work up for hereditary thrombophilia was unrevealing. Treatment with cyclophosphamide, bortezomib and dexamethasone combination regimen led to hematologic response without further thromboembolic complications. While thromboembolic complications have been reported in AL amyloidosis and cardiac involvement, this unique case highlights the complexity of the disease, the various pathogenic mechanisms at play and the difficult management decisions necessary in caring for these complex patients. A literature review of thrombembolic complications in patients with amyloidosis is presented.
一位 41 岁患有免疫球蛋白轻链(AL)系统性淀粉样变性伴心脏和胃肠道受累的女性出现多处动脉和静脉血栓栓塞并发症。用未分级肝素治疗时出现危及生命的胃肠道出血。遗传性血栓形成倾向检查无异常发现。用环磷酰胺、硼替佐米和地塞米松联合治疗方案使血液学反应得到缓解,没有发生进一步的血栓栓塞并发症。尽管 AL 淀粉样变性和心脏受累均可发生血栓栓塞并发症,但此独特病例强调了疾病的复杂性、起作用的各种发病机制以及在护理这些复杂患者时所需的困难管理决策。本文对淀粉样变性患者的血栓栓塞并发症进行了文献复习。