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KMID : 0387820150220020161
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Clinical Pediatric Hematology-Oncology
2015 Volume.22 No. 2 p.161 ~ p.166
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A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol
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Lee Jung-Won
Jung Hye-Lim
Shim Jae-Won
Kim Deok-Soo
Shim Jung-Yeon
Park Moon-Soo
Park Hee-Jin
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Abstract
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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)-α 2b and propranolol combination therapy. By the third week of IFN-α 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.
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KEYWORD
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Kasabach-Merritt syndrome, Hemangioma, Cytokine, Interferon
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