LICによる血液凝固障害を呈したクリッペルトレノネー症候群の２例

Venous Malformation and Localized Intravascular Coagulopathy: Two Cases of Klippel-Trenaunay Syndrome

はじめに

　広範囲の静脈奇形では，病変内のうっ滞した血流部位で凝固系の亢進が起こることがあり，local intravascular coagulopathy（LIC）と呼ばれ，フィブリノーゲンの消費，FDP・D-dimerの上昇を呈する 1）〜4）。LICの状態で緊急止血手術や骨折整復術を行ったクリッペルトレノネー症候群（Klippel-Trenaunay syndrome：以下，KTS）の2例を報告する。

　Activation of the coagulation system associated with venous malformations has been used to treat localized intravascular coagulopathy（LIC）. In slow-flow vascular lesions, stagnant blood leads to the production of thrombin and the subsequent conversion of fibrinogen to fibrin. LIC progresses to a systemic consumption of coagulation factors V, VII, and VIII and fibrinogen, and it results in increased FDP and D-dimers. We report two cases of coagulopathy with Klippel-Trenaunay syndrome. Both patients needed management for a wound or fracture under severe bleeding due to LIC. The current knowledge of pathophysiology, clinical laboratory findings, and patient management is also summarized.