A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol
10.15264/cpho.2015.22.2.161
- Author:
Jung Won LEE
1
;
Hye Lim JUNG
;
Jae Won SHIM
;
Deok Soo KIM
;
Jung Yeon SHIM
;
Moon Soo PARK
;
Hee Jin PARK
Author Information
1. Department of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea. hl.jung@samsung.com
- Publication Type:Case Report
- Keywords:
Kasabach-Merritt syndrome;
Hemangioma;
Cytokine;
Interferon
- MeSH:
Chemokine CCL2;
Cytokines;
Diagnosis;
Female;
Fibroblast Growth Factor 2;
Follow-Up Studies;
Hemangioma;
Humans;
Hyperemia;
Infant, Newborn;
Interferon-alpha;
Interferons;
Kasabach-Merritt Syndrome;
Prednisone;
Propranolol;
Recurrence;
Thigh;
Vascular Endothelial Growth Factor A
- From:Clinical Pediatric Hematology-Oncology
2015;22(2):161-166
- CountryRepublic of Korea
- Language:English
-
Abstract:
Kasabach-Merritt syndrome (KMS) is a rare, life-threatening disease characterized by rapidly enlarging hemangioma and consumptive coagulopathy. We report a case of KMS in a 28-day-old female neonate with a huge mixed type hemangioma on her right thigh with muscle involvement and severe venous engorgement, who was refractory to prednisone therapy, but was successfully managed with the interferon (IFN)-alpha 2b and propranolol combination therapy. By the third week of IFN-alpha 2b treatment, hematological parameters had normalized and the hemangioma size had dramatically decreased, and after 5 months of the treatment, complete resolution was observed visually. We also measured serum levels of cytokines including vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), monocyte chemoattractant protein-1 (MCP-1) and platelet-derived growth factor-BB (PDGF-BB), at diagnosis and serially during treatment but the levels did not correlate with the clinical response. The patient has not shown relapse after 20 months of follow up.
