Callander N, Rapaport S I
Department of Medicine, University of California, School of Medicine, San Diego, La Jolla.
West J Med. 1993 Apr;158(4):364-71.
We report 4 cases of Trousseau's syndrome, in which spontaneous recurrent or migratory venous thromboses, arterial emboli caused by nonbacterial thrombotic endocarditis, or both, develop in a patient with a recognized or occult malignant tumor. The clinical course of 3 of the patients emphasizes a key point: The occurrence for no known reason of thromboses preventable by anticoagulation therapy with heparin but not with warfarin sodium should alert a physician to focus diagnostic efforts on uncovering an underlying malignant lesion. Thromboses may occur months to years before the tumor is discovered, and a thorough negative initial examination does not obviate the need for a continuing search. Patients with Trousseau's syndrome have persistent low-grade intravascular coagulation, and therapy with heparin should be continued indefinitely. Stopping heparin therapy for even a day may permit a new thrombosis to develop. Immunostaining a biopsy specimen from 1 patient provided evidence that 2 properties of a neoplastic lesion are required for the syndrome to develop: The malignant cells express surface membrane tissue factor, and structural features of the tumor permit the malignant cells or vesicles it sheds to be exposed to circulating blood.
我们报告了4例特鲁索综合征病例,在这些病例中,已确诊或隐匿性恶性肿瘤患者会出现自发性反复或游走性静脉血栓形成、非细菌性血栓性心内膜炎所致的动脉栓塞,或两者皆有。3例患者的临床病程突出了一个关键点:出现可通过肝素抗凝治疗预防但华法林钠无法预防的不明原因血栓形成,应提醒医生将诊断重点放在发现潜在恶性病变上。血栓形成可能在肿瘤被发现前数月至数年出现,初次全面检查结果为阴性并不能排除持续排查的必要性。特鲁索综合征患者存在持续性轻度血管内凝血,肝素治疗应持续进行,无期限限制。即使停用肝素治疗一天也可能导致新的血栓形成。对一名患者的活检标本进行免疫染色提供了证据,表明该综合征的发生需要肿瘤病变具备两个特性:恶性细胞表达表面膜组织因子,且肿瘤的结构特征使恶性细胞或其脱落的囊泡能够接触循环血液。