Retrospective analysis in 13 children with Kasabach-Merritt phenomenon and review of literature
10.3969/j.issn.1000-3606.2017.06.015
- VernacularTitle:儿童卡梅现象13例回顾性分析并文献复习
- Author:
Yamei ZHAO
;
Yijin GAO
;
Ying ZHOU
;
Jing MA
;
Ci PAN
;
Jingyan TANG
- Keywords:
Kasabach-Merritt phenomenon;
vascular tumor;
thrombocytopenia;
sirolimus
- From:
Journal of Clinical Pediatrics
2017;35(6):458-461,466
- CountryChina
- Language:Chinese
-
Abstract:
Objective To improve understanding of the clinical manifestations, diagnosis and treatment of childhood Kasabach-Merritt phenomenon (KMP). Methods The clinical data of 13 patients admitted for KMP to XXX from January 2010 to January 2016 was retrospectively analyzed, with a review of relevant literature. Results The patients were 10 males and 3 females. The age of presentation varied from newborn to 5 months. 12 patients had cutaneous manifestations, like petechiae, ecchymosis, jaundice, skin masses, etc, 1 patient had pleural effusion. The location of lesions varied. The laboratory hallmark consists of profound thrombocytopenia and hypofibrinogenemia with elevated D-dimers. The median time from initial presentation to diagnosis was 60 days. After approaches like surgery, corticosteroids, propranolol, interferon, sirolimus, etc, 10 patients got remission while 3 patients died. 6 patients treated with sirolimushad complete response. Conclusions KMP is characterized with vascular tumor, severe thrombocytopenia and consumptive coagulopathy. Clinically, KMP often presents with early-onset and delay in diagnosis. Surgery is an effective approach for KMP. Sirolimus appears to be a promising treatment for KMP.
