达比加群成功治疗广泛血管畸形导致的消耗

病例报道:

Successfultreatmentwithdabigatranforconsumptivecoagulopathyassociatedwithextensivevascularmalformations

达比加群成功治疗广泛血管畸形导致的消耗性凝血病

AtsushiYasumoto,RyoheiIshiura,MitsunagaNarushimaandYutakaYatomia

BloodCoagulationandFibrinolysis,28:–

一名17岁日本女性患者患有广泛的静脉/淋巴管畸形,具体涉及到主动脉旁静脉曲张和躯干、腹腔脏器的淋巴结,并因此继发消耗性凝血病和重度血小板减少症(50×/L)。该患者因此参与了一项应用普萘洛尔口服治疗卡梅现象(Kasabach-Merrittphenomenon)的临床试验,但患者的血小板计数并没有恢复正常。患者还接受了静脉畸形切除术、局部止血和整形处理,补充新鲜冰冻血浆和浓缩凝血因子XIII(FXIII),还使用了甲磺酸萘莫司他和重组血栓调节蛋白进行抗凝治疗,但患者的血小板计数和纤维蛋白原水平仍未恢复正常。出血停止后,患者再没有任何药物,包括抗凝药。

A17-year-oldJapanesegirlwithconsumptivecoagulopathyandprofoundthrombocytopenia(5.0×/l)secondarytoextensivevenous/lymphaticvascularmalformationsinvolvingthepara-aorticvaricosityandlymphnodesofthetrunkandtheabdominalvisceraparticipatedinaclinicaltrialexaminingthetherapeuticefficiencyoforalpropranololforKasabach–Merrittphenomenon,butnormalizationoftheplateletcountwasnotachieved.Shehadalsoundergoneanticoagulanttherapieswithnafamostatmesilateandre







































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