婴儿期的卡萨巴赫-梅里特综合征。

Kasabach-Merrit syndrome in infants.

作者信息

Sarihan H, Mocan H, Abeys M, Akyazici R, Cay A, Imamoğlu M

机构信息

Department of Pediatric Surgery, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey.

出版信息

Panminerva Med. 1998 Jun;40(2):128-31.

PMID:9689834

Abstract

METHODS

Four infants with Kasabach-Merrit syndrome syndrome have been treated at the University Hospital, Trabzon. They had large varied-site cutaneous hemangiomas. Diagnosis was performed with clinical and laboratory studies. All patients has severe thrombocytopenia and anemia. Fibrinogen and fibrin split products were examined in two patients and lower fibrinogen and over fibrin split products levels were detected in them. All patients were admitted to the intensive care unit and they were treated with antibiotics, fresh blood transfusion and thrombocyte suspension. Two out of four patients were previously treated with steroids unsuccessfully and one patient died due to disseminated intravascular coagulopathy. Three patients were treated with interferon alfa-2a and compression.

RESULTS

In two patients the lesions regressed 60-80% following the five months therapy and in the other patient the lesion was completely excised after one month therapy.

CONCLUSIONS

Interferon alfa-2a and compression were found to be remarkably effective in the treatment of Kasabach-Merrit syndrome.

摘要

方法

特拉布宗大学医院治疗了4例患有卡萨巴赫 - 梅里特综合征的婴儿。他们患有大小和部位各异的皮肤血管瘤。通过临床和实验室研究进行诊断。所有患者均有严重血小板减少症和贫血。对2例患者检测了纤维蛋白原和纤维蛋白降解产物，发现他们的纤维蛋白原水平较低且纤维蛋白降解产物水平过高。所有患者均入住重症监护病房，接受抗生素、新鲜输血和血小板悬液治疗。4例患者中有2例先前接受类固醇治疗但未成功，1例患者因弥散性血管内凝血死亡。3例患者接受了α-2a干扰素治疗和压迫治疗。