Klippel-Trénaunay-Weber Syndrome Complicated by DIC Associated with Bone Fracture
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Klippel-Trénaunay-Weber syndrome is characterized by three major abnormalities; hemangioma of the nevus flammeus type, varicose veins, and hypertrophy of soft tissue and bone. These abnormalities usually affect a single extremity. In this paper, we describe a 23-year-old man with this syndrome complicated by disseminated intravascular coagulation (DIC) which was triggered or exacerbated by fracture of the right lower leg.Large ecchymosis from intramuscular injection sites, bleeding tendency from a puncture site of the right femoral artery, and a positive test for urinal occult blood were noted. Femoral arteriograph revealed many hemangiomas in the muscles surrounding fractured bones. Laboratory studies showed that prothrombin time was 13.0s (control 10.6s), fibrinogen 25mg/dl, antithrombin III 101%, FDP 95μg/ml, D-D dimer 33μg/ml, plasmin-α2 plasmin inhibitor complex (PIC) 14μg/ml, thrombin-antithrombin III complex 60.0ng/ml, and platelet counts 13.6×104/μl. These findings are compatible with DIC. Severe hypofibrinogenemia without thrombocytopenia suggested that primary fibrinolytic state may also coexist with DIC. Tissue damages of hemangioma associated with bone fracture may release activators of both coagulation and fibrinolytis, resulting in DIC and primary hyperfibrinolysis.Continuous infusion of heparin and replacement therapy with fresh frozen plasma (FFP) were started. With these treatments, DIC and primary hyperfibrinolytic state were controlled to the extent that the patient did not have obvious bleeding tendencies, even without amputation of the involved limb.
- 一般社団法人 日本血栓止血学会の論文
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