Trousseau症候群

Trousseau症候群
1985年トルソーによって悪性腫瘍と静脈血栓との関連が最初に報告されている。
古くは悪性腫瘍に関連して移動性表在性血栓性静脈炎と有痛白股腫の起こる疾患群といわれていたが、現在では、凝固異常によって引き起こされる塞栓症を総称して呼んでいるようである。

1985年のGrausらによる大規模剖検調査によると，悪性腫瘍患者3,426例のう ち，500例（14.6％）に脳血管障害を認め，そのうち244例は脳出血，256例が脳梗塞であったという。脳梗塞の117 例が症候性脳梗塞であり，悪性腫瘍患者の合併症として脳血管障害は，高頻度に認められるといえる。
　悪性腫瘍に伴う脳血管障害の原因はさまざまで ある。潜在性の悪性腫瘍の遠隔効果により神経症状を生じるTrousseau症候群は，血液凝固亢進により脳卒中症状を生じる病態である


* Migratory superficial thrombophlebitis (Trousseau's syndrome)
* Idiopathic deep venous thrombosis and other venous thrombosis
* Non bacterial thrombotic endocarditis (marantic endocarditis)
* Disseminated intravascular coagulation (DIC)
* Thrombotic microangiopathy (eg, hemolytic-uremic syndrome)
* Arterial thrombosis

found evidence of thrombosis in 30 percent of patients who died of pancreatic cancer; the incidence was over 50 percent in those with tumors in the body or tail of the pancreas [6].

. If a tumor is discovered, it is usually an adenocarcinoma. In one review of patients with Trousseau's syndrome, the following associated tumors were seen [4]:


Pancreas — 24 percent
Lung — 20 percent
Prostate — 13 percent
Stomach — 12 percent
Acute leukemia — 9 percent
Colon — 5 percent


This syndrome occurs in up to 10 percent of patients with pancreatic carcinoma. Treatment is difficult; heparin can relieve some of the manifestations, while coumadin appears to be without effect [15,16].

Mucin — Mucins produced by adenocarcinomas may trigger this syndrome by reacting with leukocyte and platelet selectins, resulting in the production of platelet-rich microthrombi [17,18]. As an example, one study has shown that, while thrombotic risk was increased 20-fold in patients with lung cancer, the relative risk of venous thrombosis was significantly higher in those with (mucin-containing) adenocarcinoma than in the squamous cell variant (hazard ratio 3.1; 95% CI 1.4-6.9) [19]. (See "Pathology of lung malignancies", section on 'Adenocarcinoma'.)

Heparin has the property of blocking selectin recognition of ligands, a property not shared by vitamin K antagonists. This may explain the superior efficacy of heparin in this setting [18]. (See "Treatment of venous thromboembolism in patients with malignancy", section on 'LMW heparin versus warfarin'.)
ヘパリンが効果的でワーファリンは適切ではない
Trousseau's syndrome. Devastating coagulopathy in the absence of heparin.

Bell WR, Starksen NF, Tong S, Porterfield JK.
Abstract

Two patients with Trousseau's syndrome experienced frequently recurring concomitant arterial and venous thrombotic events that resulted in sequential amputation and loss of the lower extremities. Serial examination of the blood in the patients demonstrated that these devastating thrombotic events were preceded by severe disseminated intravascular coagulopathy that occurred within an interval of a few hours. Warfarin therapy was without effect in preventing the occurrence of these events. Both patients demonstrated the absolute need for intravenous heparin, which effectively prevented the thrombotic events; when it was discontinued, the immediate consequences were disastrous and resulted in death. Techniques for long-term outpatient heparin therapy are discusse

VENOUS THROMBOEMBOLISM — The majority of cancers associated with thromboembolic events are clinically evident and have been previously diagnosed at the time of the event. However, some patients with venous thromboembolism (VTE) have an occult malignancy which is not diagnosed until many months following the event.

Known malignancy — The risk factors for VTE in patients with known malignancy were evaluated in a Dutch population-based case-control study of 3220 patients with a first DVT of the leg and/or pulmonary embolism and 2131 controls. Results of this study included the following [20]:


The overall risk of VTE was significantly