Retrospective analysis in 13 children with Kasabach-Merritt phenomenon and review of literature / 临床儿科杂志
Journal of Clinical Pediatrics
;
(12): 458-461,466, 2017.
Article
in Chinese
| WPRIM
| ID: wpr-619025
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Language:
Chinese
Journal:
Journal of Clinical Pediatrics
Year:
2017
Type:
Article
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