Clinicopathological analysis of a case of adult Kasabach-Merritt syndrome / 中华皮肤科杂志

ABSTRACT

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.