CAJ | 학술논문

患者女,52岁,不明原因血小板减少29年、右面颊部紫红色斑块9年.给予糖皮质激素等治疗后病情稍有好转,但血小板从未上升至正常,糖皮质激素减量后长期服中药调理.近来患者面颊部斑块肿胀疼痛,血小板进行性下降.血常规检查白细胞3.0 × 109/L,血红蛋白85 g/L,血小板2×109/L.CT检查示肝内多发血管瘤,其中肝左外叶1个14 cm×5.7 cm.内科治疗后手术切除肝左外叶巨大血管瘤.术后血小板上升至120×109/L,面颊部肿胀消失.术后病理诊断:肝脏巨大海绵状血管瘤,血管腔内广泛血栓形成伴机化.综合分析,本例诊断:成人Kasabach-Merritt综合征.
환자녀,52세,불명원인혈소판감소29년、우면협부자홍색반괴9년.급여당피질격소등치료후병정초유호전,단혈소판종미상승지정상,당피질격소감량후장기복중약조리.근래환자면협부반괴종창동통,혈소판진행성하강.혈상규검사백세포3.0 × 109/L,혈홍단백85 g/L,혈소판2×109/L.CT검사시간내다발혈관류,기중간좌외협1개14 cm×5.7 cm.내과치료후수술절제간좌외협거대혈관류.술후혈소판상승지120×109/L,면협부종창소실.술후병리진단:간장거대해면상혈관류,혈관강내엄범혈전형성반궤화.종합분석,본례진단:성인Kasabach-Merritt종합정.
A 52-year-old female presented with unexplained thrombocytopenia for 29 years and a prunosus plaque on the right cheek for 9 years.She had ever been treated with glucocorticoids,which resulted in a slight improvement of the condition,but the platelet count remained below the normal range.During glucocorticoid tapering,she had administrated traditional Chinese medicines for a long peroid.Recently,the plaque on the cheek became swollen and painful with a progressive decrease in platelet count.Routine blood test showed that the count of white blood cells and platelets was 3.0 × 109/L and 2 × 109/L respectively,and the concentration of hemoglobin was 85 g/L.Computed tomography (CT) scan revealed multiple liver hemangiomas,one of which in the left liver lobe measured 14 cm × 5.7 cm in size.After medical treatment,the giant hemangioma in the left lobe was resected.Thereafter,the platelet count increased to 120 × 109/L with the subsidence of swelling of the right cheek.Postoperative pathological examination confirmed a diagnosis of giant cavernous hemangioma of the liver with extensive intravascular thrombosis and thrombus organization.Based on these findings,the case was diagnosed as adult Kasabach-Merritt syndrome.