Abstract
Kasabach-Merritt phenomenon (KMP) is relatively rare in childhood and adolescents with high mortality rate because of its hemorrhagic complications and unresponsiveness to treatments such as corticosteroids, vincristine, intravascular embolization, and/or surgery. Propranolol, a β-adrenergic receptor blocker, has a promising efficacy against vascular tumors such as infantile hemangiomas. But limited and variable data has been reported regarding the role of propranolol in treatment of KMP. We herein reported the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with kaposiform hemangioendothelioma with successful treatment of both clinical and hematologic responses. After eight months of follow-up, patient still had stable cutaneous lesion while receiving propranolol monotherapy. Regular hematologic monitoring was done in order to detect any late relapse of the disease. Six months after discontinuation of propranolol, patient has still remained free of hematologic relapse, and primary cutaneous lesion has become a pale pink, 1 cm sized skin lesion.
Highlights
Kasabach-Merritt phenomenon (KMP) is characterized by a vascular tumor called kaposiform haemangioendothelioma (KHE), with evidence of thrombocytopenia, hypofibrinogenaemia, and/or coagulopathy
Propranolol is a nonselective β-adrenergic receptor blocking agent that has a promising efficacy against vascular tumors such as infantile haemangiomas (IHs) [5,6,7]
We demonstrated the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with successful treatment of both clinical and hematologic responses
Summary
Kasabach-Merritt phenomenon (KMP) is characterized by a vascular tumor called kaposiform haemangioendothelioma (KHE), with evidence of thrombocytopenia, hypofibrinogenaemia, and/or coagulopathy. In 2011, a case report described the effectiveness of oral propranolol in combination with four doses of weekly intravenous vincristine in treatment of KHE with KMP [8]. In a later report by Chiu et al [9] in a case series of six KHE with KMP, only one out of four patients showed responsiveness (normalization of hematologic parameters of KMP) to propranolol used in combination with prednisolone, either with or without vincristine, while one out of two patients showed responsiveness to propranolol monotherapy. We demonstrated the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with successful treatment of both clinical and hematologic responses. Patient has still remained free of hematologic relapse, and primary cutaneous lesion has become a pale pink, 1 cm sized skin lesion
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