Clinical analysis of kasabach-merritt syndrome in 17 neonates
نویسندگان
چکیده
BACKGROUND Kasabach-Merritt syndrome (KMS) is characterized by giant hemangiomas and severe thrombocytopenia, which may result in life-threatening multi-organ hemorrhage. This study evaluated the clinical characteristics, treatments, and outcomes in neonates with KMS, in order to find out the optimal therapy. METHODS The clinical data of 17 patients treated for KMS in the Department of Neonates, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, China from January 2007 to January 2012 were retrospectively analyzed. RESULTS The patients were 13 males and 4 females, aged 17 hours to 28 days at admission. Four patients had visceral hemangiomas and 13 had cutaneous hemangiomas. All had thrombocytopenia and coagulation disorders. Intravenous steroid therapy was initially effective in 6 patients (of which 3 relapsed) and ineffective in 11. The 11 patients with a poor response to steroids and the 3 who relapsed underwent arterial embolization therapy, which was effective in 9 patients (of which 1 relapsed), ineffective in 4, and discontinued before completion in 1. Subsequently, four patients in whom arterial embolization therapy was ineffective and one with relapse were treated with vincristine. This was effective in four patients, and the other died of disseminated intravascular coagulation. Steroid therapy was effective in 35.3% of patients, but the relapse rate was 50%. Arterial embolization was effective in 64.3% of patients and vincristine was effective in 80%. CONCLUSIONS In patients with neonatal KMS, steroid therapy has a low rate of effectiveness and high rate of relapse. Arterial embolization has a good rate of effectiveness. Combined steroid and embolization therapy should be considered for first-line treatment of neonatal KMS. If this approach is ineffective, vincristine may be useful.
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A new successful combination therapy with atenolol and prednisolone for Kasabach-Merritt syndrome
Kasabach-merritt syndrome is a rare life-threatening syndrome associated with vascular tumors such as tufted angioma and kaposiform hemangioendothelioma. For this syndrome, there exist a few treatment modalities with variable results. For many years, corticosteroids have been employed in the first-line therapy. Nowadays, on the other hand, β blockers such as propranolol have been used owing to ...
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Haemangioma is a common type of vascular tumour in paediatric age group [1]. Most of them are benign and non-life threatening [1]. Kasabach Merritt syndrome (KMS) is a combination of giant haemangioma, thrombocytopenia and consumptive coagulopathy [1-5]. It presents with diverse clinical presentation and may be a part of other recognized syndrome [1,2]. KMS is a well-recognized but rare clinica...
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